A Retrospective Analysis of Tear Film and Blink Parameters in Patients With Ocular Surface Disease.

OBJECTIVES: We had two objectives: (1) to investigate differences in tear film and blink parameters in patients with six ocular surface diseases (OSD): ocular graft versus host disease (oGVHD), Stevens-Johnson syndrome (SJS), lax eyelid syndrome, meibomian gland dysfunction (MGD), obstructive sleep apnea, and dry eye disease; and (2) to characterize the relationships between lipid layer thickness (LLT), meiboscore, Schirmer I test, and three blink parameters: partial blink rate (PBR), interblink interval (IBI), and interpalpebral fissure height (IPFH) in this group of patients. METHODS: This is a single-site retrospective analysis of 228 patients (456 eyes) with six different primary OSD at Loyola University Medical Center between January 2018 and July 2020. Objective parameters included: LLT, IBI, PBR, IPFH, Pult meiboscore, and Schirmer I score. Linear mixed effects models were used to analyze the relationships between these parameters. RESULTS: The meiboscore was highest in patients with SJS compared with the other five groups. There was significant variability in IPFH across different groups, with patients with oGVHD having the smallest height. An increase in IPFH was associated with increases in IBI, PBR, and Schirmer score. Patients with no partial blinks demonstrated a statistically significant higher IBI than patients who showed 1%-50% partial blinks. Finally, no statistically significant relationship was found between the following: (1) meiboscore and LLT, (2) OSDI and IPFH, (3) Schirmer score and IBI, or (4) LLT and IBI. CONCLUSIONS: There were fewer than anticipated differences in objective tear film and blink parameters between patients with six different OSD. A statistically significant association was found between IPFH and Schirmer score and IPFH and IBI, representing new findings in tear film research. Further investigation of the relationship between IPFH and IBI is needed.

Autoimmune retinopathy associated with systemic autoimmune disease: a case series.

OBJECTIVE: To describe various presentations of autoimmune retinopathy (AIR) associated with systemic autoimmune diseases. DESIGN: Case series. PATIENTS AND METHODS: Four patients with systemic autoimmune disorders and AIR are described in this report. The clinical and multimodal imaging characteristics, systemic work-up, genetic testing results, management, and course of disease are detailed. RESULTS: The multimodal retinal features of 4 cases of AIR including the findings of fundus autofluorescence, optical coherence tomography, and electrophysiology necessary to document progressive photoreceptor loss are described. Each case of AIR was associated with a complicated autoimmune disorder. Case 1 was associated with chronic inflammatory demyelinating polyneuropathy and showed marked improvement with systemic steroid and intravenous immunoglobulin therapy. Case 2 was associated with rheumatoid arthritis, and the AIR condition progressed despite systemic immune therapy. Case 3 was associated with Lambert-Eaton myasthenic syndrome, and AIR developed 6 years later and stabilized with systemic immune therapy. Case 4 was associated with necrobiotic xanthogranuloma followed by AIR and was managed by systemic immune therapy. CONCLUSIONS: AIR in association with these systemic conditions is rarely reported. Our cases highlight the gaps in our current understanding of the definition, systemic associations, pathogenesis, and management of AIR and the importance of multimodal imaging and a multidisciplinary approach in managing patients with suspected AIR.

32-year-old diabetic patient with progressive vision loss and crystalline retinopathy.

PURPOSE: To report the case of severe bilateral retinal vascular occlusion in a patient with hyperoxalosis and chronic renal failure. METHODS: Observational case report. Medical and imaging records were retrospectively reviewed. The patient was imaged with ultra-widefield (UWF) fundus photography and fluorescein angiography (UWF-FA), cross sectional and en face spectral-domain optical coherence tomography (SD-OCT), and OCT angiography. RESULTS: A 32-year-old diabetic patient receiving peritoneal dialysis was referred because of severe vision loss. UWF color fundus photography showed diffuse sclerotic retinal vessels and diffuse intraretinal crystals in both eyes. UWF-FA illustrated near-complete retinal vascular occlusion and capillary wipe out in both eyes. SD-OCT demonstrated diffuse inner and middle retina thinning in both eyes and multiple intraretinal hyperreflective foci consistent with crystalline deposits in all retina layers of both eyes. OCT angiography revealed severe capillary and large vessel non-perfusion in the superficial and deep retinal capillary plexus of each eye. The serum oxalate levels were increased at 28 µmol/L (reference range < 2 µmol/L) and genetic testing was positive for a heterozygous mutation of the AGXT (Alanine-Glyoxylate Amino Transferase) gene that causes type 1 autosomal recessive primary hyperoxaluria. CONCLUSION: A diagnosis of hyperoxalosis causing severe retinal vascular occlusion was rendered. Hyperoxalosis was the result of multiple factors including heterozygous AGXT mutation, chronic renal failure insufficiently treated with peritoneal dialysis, and a diet high in oxalate. This case highlights the importance of ruling out retinal oxalosis in patients on peritoneal dialysis in order to initiate prompt hemodialysis and prevent severe retinal vascular occlusion.

Visualization of Photoreceptors in Birdshot Chorioretinopathy Using Adaptive Optics Scanning Laser Ophthalmoscopy: A Pilot Study.

PURPOSE: Adaptive optics scanning laser ophthalmoscopy (AOSLO) allows en face visualization of specific layers of the retina. This pilot study evaluated the ability of AOSLO to visualize photoreceptor integrity in patients with birdshot chorioretinopathy (BCR). METHOD: A total of 16 consecutive patients with HLA-A29+ BCR were imaged using the prototype Apaeros retinal imaging system. Images of high quality were aligned with infrared reflectance photos and correlated with spectral domain optical coherence tomography (SD-OCT). RESULTS: Images of four eyes of three patients were of sufficient quality to allow posterior pole montage and point-to-point correlation with SD-OCT. Areas of photoreceptor disruption on SD-OCT were seen as patchy areas of loss on AOSLO, whereas areas of intact interdigitation zone and inner segment/outer segment junction correlated with normal appearing photoreceptors on AOSLO. CONCLUSIONS: Using AOSLO, we found one instance of subclinical photoreceptor disruption not seen on SD-OCT. Ultimately, there are unique challenges associated with imaging BCR patients using AOSLO.