A 51-year-old homeless Caucasian male with a history of posttraumatic stress disorder, major depressive disorder, anxiety, bipolar disorder, hypothyroidism, and extensive history of IV drug use was transferred to our hospital for the management of right upper and lower extremity abscesses, right facial swelling, purulent nasal drainage, fever with chills, and nausea for a duration of 3 weeks. The patient initially attempted to drain an abscess on his right lower extremity using an unsterile needle, which eventually developed into multiple abscesses on both his right-upper and right-lower extremities. The abscesses also involved his face with continuous purulent drainage, prompting his decision to seek medical care. Per documentation from an outside facility, the patient had an intact vision and normal extraocular movements with no history of headache, eye pain, facial numbness, difficulty breathing, dysphagia, vomiting, or cough. There was no pertinent surgical or family history. Social history was significant for alcohol abuse, tobacco, illicit IV drug use (heroin and amphetamine), and noncompliance with medications.
Vitals on admission showed a temperature of 37.7  C, a pulse of 117 beats per minute, a respiratory rate of 22 cycles per minute, and blood pressure of 156/86 mm Hg with a saturation of 94% on room air. Succinct examination revealed facial erythema and swelling, purulent drainage from the nares with necrotic tissue over the nose and abscesses with purulent drainage over the right upper and lower extremities. The patient was also noted to have severe pain, conjunctival chemosis and injection with reduced vision, and limited extraocular movements in the right eye. The intraocular pressure and visual acuity could not be assessed at this time as the patient refused the exam. Emergent bedside right lateral canthotomy with cantholysis was performed due to concern for right periorbital cellulitis and orbital compartment syndrome. The patient was promptly started on acetazolamide 250 mg IV every 6 hours for 4 doses, brimonidine 0.15% ophthalmic solution twice a day, and timolol 0.5% eye drops twice a day to help reduce intraocular pressure.
Pertinent laboratory workup showed leukocytosis of 43.3 x 103/UL with an absolute neutrophil count of 39.36 x 103/UL and bands of 6.47 x 103/UL; anemia with hemoglobin and hematocrit of 9.0 g/dL and 26.7%; platelet count of 507 x 103/UL; an unremarkable comprehensive metabolic panel, lactic acid of 3 mmol/L, erythrocyte sedimentation rate of 21 mm/hr, and C-reactive protein was >18 mg/dL. The urine drug screen was positive for opioids and amphetamines, the hepatitis panel was reactive for hepatitis C, and a hemoglobin A1c of 6.1% was noted. Computed tomography (CT) scan of the face and orbits (Figure 1) showed marked fat stranding, soft-tissue thickening, and edema of the periorbital nasal bridge and right nasal bone. No preseptal orbital abscess or foreign body was displayed.
The patient underwent urgent incision, drainage, and debridement of the abscesses and was started on broad-spectrum IV antibiotic coverage with clindamycin 900 mg every 6 hours and piperacillin-tazobactam 4.5 g every 6 hours. This regimen was eventually changed to IV vancomycin after blood and wound cultures grew methicillin-resistant Staphylococcus aureus (MRSA). On day 3 of admission, the patient developed a markedly elevated white blood cell count of 70.4 x 103/UL and a significant drop in hemoglobin to 6.4g/dL despite appropriate antibiotic coverage. He developed septic and hemorrhagic shock with a blood pressure of 61/36 mm Hg. The patient was intubated and stabilized with blood transfusions and pressor support. An endoscopy was performed and revealed a bleeding gastric ulcer that was clipped.
One week after the patient was extubated, he reported right-sided vision loss. A comprehensive eye exam was performed, which revealed an intraocular pressure of 15 mm Hg, a round, reactive pupil with a relative afferent pupillary defect, negative for retropulsion, and a full range of extraocular movements with mild pain on abduction secondary to the lateral canthotomy. Visual fields by confrontation showed a central and peripheral scotoma, and a visual acuity of 20/light perception vision was noted. Direct ophthalmoscopy displayed lateral canthal hyperemia, mild purulent discharge, trace temporal conjunctiva injection with normal epithelium, endothelium, stroma, tear film, corneal sensation, and an intact blink reflex. The anterior chamber had normal depth without cells or flare; the iris was normal with no rubeosis, and the lens was normal. No lymphadenopathy was appreciated in the preauricular group of lymph nodes. Indirect ophthalmoscopy showed a clear vitreous with no cells, blurred margins of the optic nerve head, mild attenuation of exiting vessels, and a cup-to-disk ratio of 0.1. Fundoscopy further revealed attenuated arteries with a cartwheel appearance, focal arterial sheathing, large artery yellow plaques, aneurysmal dilation-beading of vessels in the far periphery at 11 and 1 O'clock, respectively; inferior arcade-peripapillary vein flame hemorrhage, and superior arcade paramacular flame hemorrhage with few scattered Roth spots. Diffuse retinal whitening, cherry-red spot, and multiple dot-blot hemorrhages were seen predominantly in the superior lattice. Evidently, the findings were consistent with both central retinal artery and vein occlusion (Figure 2). The left eye exam was grossly normal.
Given the unilateral loss of vision, a magnetic resonance imaging (MRI) scan of the brain and a carotid artery Doppler analysis were performed to rule out concurrent stroke:both of which were negative. The MRI reflected the bulging of the optic disc and thickening of the right optic nerve as seen in Figure 3. An echocardiogram was performed to rule out intracardial shunts. The patient was also worked up for hypercoagulable states and autoimmune disorders due to the exceptionally rare finding of CCRAVO. The patient had a normal activated prothrombin time (aPTT) and was negative for anti-thrombin III antigen, protein C, protein S, activated protein C resistance, factor V Leiden mutation, prothrombin G20210A mutation, hyperhomocysteinemia, leukocyte alkaline phosphatase deficiency, antinuclear antibody (ANA), rheumatoid factor (RF), lupus anticoagulant, and anti-cardiolipin. No monoclonal proteins were detected on serum protein electrophoresis. The infectious workup was negative for treponemal serology and HIV.
During the hospital course, the patient was also noted to have multiple episodes of desaturation on room air to 70% to 80% saturation. Computed tomography of the thorax revealed ill-defined ground-glass opacities, interstitial thickening, and a thick-walled cystic lesion. Fungal infection, tuberculosis, and septic emboli secondary to MRSA sepsis were considered differentials. Work-up for the same was negative for tuberculosis by QuantiFERON Gold and sputum culture, coccidioides, and aspergillus. Hence, respiratory failure was attributed to septic emboli which improved significantly with treatment.
The patient received a 6-week course of inpatient IV vancomycin, aggressive wound care, and supportive treatment which led to the resolution of his symptoms. He was referred to a long-term acute care facility for appropriate wound care, de-addiction, and management of multiple psychiatric comorbidities. An outpatient ophthalmology referral was provided to monitor for the development of neovascular glaucoma or retinal vascularization bleeding. At discharge, the wounds had improved, no further gastrointestinal bleed was noted, and the patient was saturating >90% on room air, however, he had an irreversible vision loss in his right eye.