Concurrent primary Sjögren's syndrome and isolated ocular sarcoidosis presenting with bilateral corneal scarring and dry eye.

PURPOSE: To report the case of a patient who presents with multiple progressive ocular diseases who is diagnosed with concurrent primary Sjögren's syndrome and isolated ocular sarcoidosis. OBSERVATION: A 60-year-old woman was referred for dry eye disease, bilateral interstitial keratitis, anterior uveitis, and progressive glaucoma. There was clinical suspicion of an autoimmune etiology due to her ocular history, risk factors, and presentation. Thorough diagnostic testing revealed both primary Sjögren's syndrome and ocular sarcoidosis. After 2.5 years of systemic treatment and follow up, the patient currently remains stable. CONCLUSIONS AND IMPORTANCE: Autoimmune disease may underlie those with progressive ocular disease with an unknown etiology. More than one autoimmune disease may be the cause of ocular findings, especially for patients with a complicated presentation. Proper awareness, clinical suspicion, and diagnosis of these diseases can greatly improve a patient's condition and prevent future ocular and systemic complications.

Case series: Delayed diagnoses of Acanthamoeba keratitis.

PURPOSE: To describe two cases of Acanthamoeba keratitis in contact lens wearers misdiagnosed as Herpes simplex keratitis. OBSERVATIONS: Case 1 is a 54-year-old male that was misdiagnosed with Herpes simplex keratitis who developed advanced Acanthamoeba keratitis. His treatment course was complex and involved both medical therapy and surgical intervention with poor resolution. Case 2 is an 18-year-old male who was also initially misdiagnosed and treated for Herpes simplex keratitis, but ultimately treated with polyhexamethylene biguanide and chlorhexidine with complete resolution. CONCLUSIONS AND IMPORTANCE: The clinical presentation of Acanthamoeba keratitis may closely resemble other causes of keratitis and continues to be misdiagnosed, leading to delayed diagnosis and treatment. However, given the significant morbidity and challenging treatment course for Acanthamoeba keratitis, it is important for clinicians to maintain a high suspicion for Acanthamoeba and to consider obtaining cultures in contact lens wearers with atypical keratitis prior to making a diagnosis of Herpes simplex keratitis.

Aspiration of Tri-folded, Endothelium-In Grafts for Descemet Membrane Endothelial Keratoplasty.

PURPOSE: In Descemet membrane endothelial keratoplasty (DMEK), loading and positioning of tri-folded grafts into a cartridge is generally conducted with forceps or a hook, risking graft tear or trauma. We demonstrate the feasibility of loading tri-folded grafts into a cartridge with no touch to the endothelium required beyond the tri-folding process. METHODS: A corneoscleral rim with a prestripped DMEK graft is placed into a petri dish. After the graft is tri-folded with forceps and removed from its stromal attachment, the graft is gently wicked into the tip of a saline-filled Alcon B IOL cartridge connected to IV extension tubing and a 3 cc syringe, drawn into the cartridge by positioning it adjacent to the graft tip. The remainder of the graft is aspirated with the addition of saline. The cartridge orientation is reversed for graft injection. In this retrospective analysis, we analyzed surgical videos for preparation times, and assessed postoperative visual acuity, pachymetry, and endothelial cell density. RESULTS: Thirteen cases underwent this approach. Median preparation time from stain to cartridge eye contact was 8.5 minutes, and time from graft injection to final centration and bubbling was 2.9 minutes. Corneal thickness decreased from a median of 623 microns preoperatively to 566 µm at 1 month (P = 0.038). Visual acuity improved by 1 month by a median of 0.3 logarithm of the minimum angle of resolution (logMAR) (P = 0.016). Endothelial cell density decreased by 32.4% at 1 month compared with baseline. CONCLUSIONS: Endothelium-in DMEK grafts may be loaded into a plastic cartridge using a skill set similar to aspiration of a scroll.

Vascular Steal Syndrome, Optic Neuropathy, and Foreign Body Granuloma Reaction to Onyx-18 Embolization for Congenital Orbito-Facial Vascular Malformation.

A 34-year-old patient presented with a right orbito-facial mass since childhood, consistent with a congenital arteriovenous (AV) malformation. Prior to presentation, she had multiple incomplete surgical resections and embolizations with N-butyl acetyl acrylate and Onyx-18. The patient reported gradual, progressive vision loss shortly after Onyx-18 embolization. Five months after embolization, she presented with decreased vision, disfigurement and mechanical ptosis relating to a large subcutaneous mass affecting the medial right upper eyelid and forehead. Significant exam findings included a visual acuity of 20/400 (20/60 prior to embolization), an afferent pupillary defect, and optic disc pallor. MRI and angiography revealed a persistent AV malformation with feeders from the ophthalmic artery and an absent choroidal flush to the right eye. Pathology from surgical resection showed a significant foreign body giant cell reaction to the embolization material adjacent to the vessels. We suggest that an incomplete embolization with Onyx-18 may have caused vascular steal syndrome from the ophthalmic artery.

Bilateral idiopathic optic neuritis in pregnancy.

A 33-year-old primiparous woman at 31 weeks gestation with no prior history of ocular disease, autoimmune disease, or recent viral infection presented with rapidly decreasing visual acuity to 20/400 OD and counting fingers OS over 11 days. MRI demonstrated mild bilateral optic nerve enhancement. Humphrey 30-2 visual field testing showed bilateral seco-central scotomas. MR venogram, visual evoked potentials, preeclampsia workup, and serum blood tests for folate, B6, B12, Toxoplasmosis, Bartonella, Lyme disease, ACE levels, RPR, and LFTs were all within normal limits. After one-time treatment of IV 125 mg methylprednisolone and spinal tap, visual acuity improved to 20/20 OD and 20/20 OS.