Specific postoperative complications of vitrectomy in hereditary transthyretin amyloidosis.

INTRODUCTION: Vitrectomy may improve visual acuity of hereditary transthyretin amyloidosis (ATTRv) patients presenting with vitreous opacities but is associated with severe complications. The objective of this study is to report visual outcomes, early and late complications of a series of ATTRv patients who underwent vitrectomy in the French ATTRv reference center. METHODS: This retrospective, single-center study, included all ATTRv patients who underwent vitrectomy between 2002 and 2017. Data were collected on pre and postoperative best corrected visual acuity (BCVA) and early and late postoperative complications. RESULTS: A total of 21 eyes from 15 patients were included. The mean postoperative follow-up was 40 ± 20 months (6-160 months). BCVA increased from 0.7 ± 0.4 LogMAR preoperatively to 0.3 ± 0.4 LogMAR (p = 0.003) at last postoperative visit. During follow-up, all initially glaucomatous eyes worsened, with three eyes (37%) requiring filtering surgery and two eyes (25%) had further vision loss. Among non-glaucomatous patients, four eyes (31%) developed glaucoma with two requiring trabeculectomy and one eye (8%) had further vision loss. Three eyes (three patients) presented with complications of amyloid angiopathy. Three eyes (three patients) experienced recurrence of vitreous deposits requiring surgical revision. CONCLUSION: Due to the potential complications, vitrectomy in ATTRv requires specific perioperative management and life-long postoperative monitoring.

Diffusion tensor magnetic resonance imaging of trigeminal nerves in relapsing herpetic keratouveitis.

BACKGROUND: Corneal hypoesthesia is the landmark of HSV and VZV keratitis and can lead to neurotrophic keratitis. Diffusion tensor imaging (DTI) is a new magnetic resonance imaging (MRI) derived technique, which offers possibilities to study axonal architecture. We aimed at assessing the potential impact of recurrent HSV or VZV-related keratitis on the axonal architecture of trigeminal nerves using DTI. DESIGN: Prospective non-interventional study. PARTICIPANTS: Twelve patients and 24 controls. METHODS: DTI using MRI of the trigeminal fibers and corneal esthesiometry using the Cochet-Bonnet esthesiometer were acquired for patients affected by unilateral and recurrent HSV or VZV-related keratitis (3 months after the last corneal inflammatory event), and control subjects with no history of ocular or neuronal disease affecting the trigeminal pathways. MAIN OUTCOME MEASURES: Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were compared between the 2 eyes of both patients and controls, and correlated with corneal esthesiometry. RESULTS: FA was lower in the trigeminal fibers ipsilateral to the affected eye compared to the non-affected side (0.39±0.02 versus 0.46±0.04, P=0.03). This difference was more important than the intra-individual variability observed in controls. Concomitantly, the asymmetry in ADC results was significantly correlated with the loss of corneal sensitivity in the affected eye. CONCLUSIONS: Corneal hypoesthesia related to HSV and VZV keratitis is associated with persistent modifications in the architecture and functionality of the trigeminal fibers. These results add further explanation to the pathogenesis of HSV and VZV-induced neurotrophic keratitis, which may occur despite an apparent quiescence of the disease.

Retinal vasculitis revealing immunoglobulin G subclass deficiency.

Immunoglobulin G (IgG) subclass deficiency is a rare primary immunodeficiency syndrome characterized by recurrent infections and autoimmune disorders. However, there have been no reports of ocular involvement, either inflammatory or infectious, in association with IgG subclass deficiency. The authors report the first case of retinal vasculitis that led to the diagnosis of IgG subclass deficiency, in a patient with a history of inflammatory bowel disease and recurrent infections of previously unknown origin.

Combination of the Schirmer I and phenol red thread tests as a rescue strategy for diagnosis of ocular dryness associated with Sjögren's syndrome.

PURPOSE: To define a combination of the Schirmer I and phenol red thread (PRT) tests that improves the screening of patients with ocular sicca syndrome. METHODS: The PRT test was performed before (PRT1) and after (PRT2) the Schirmer I test, in both eyes of 143 patients complaining of ocular dryness secondary to Sjögren's syndrome or sicca asthenia polyalgia syndrome (SAPS; 72 and 71 patients, respectively), and in 40 control patients. Groups were matched by age and sex. After determining the best cutoff values using the receiver operating characteristic procedure, several combinations of PRT and Schirmer I were assessed to improve the predictive values of the procedure. RESULTS: The best cutoff value for PRT2, estimated at 15 mm, provided a satisfying match between sensitivity and specificity indexes (68% and 90%, respectively), similar to those obtained with the Schirmer I test. If PRT1 alone was ineffective to screen SGS from control patients, the comparison between PRT1 and PRT2 (so-called "DeltaPRT") was found as a good marker to detect patients with persistent tear reflex. Interestingly, the combination of positive Schirmer I, PRT 2, and/or ΔPRT tests was found to be highly predictive of severe ocular sicca syndrome. CONCLUSIONS: The combination of the Schirmer I and PRT tests strongly improves the screening procedure to detect patients with ocular dryness related to Sjögren's syndrome or SAPS. It could be more widely used in daily clinical practice, aside from the Schirmer I test, to optimize the work-up of patients presenting with dry-eye subjective signs.

Choroidal ischemia secondary to a diving injury.

PURPOSE: To report a case of choroidal ischemia in a professional diver after a decompression sickness accident. METHODS: A 31-year-old man presented a decompression sickness accident without any systemic manifestation. The symptoms were exclusively ophthalmic with sudden decrease of vision and presence of scotoma predominantly on the left eye immediately after a dive. The patient had a complete clinical ophthalmologic examination as well as fluorescein angiography, optical coherence tomography, central visual field, visual-evoked potentials, and electroretinogram examinations that were repeated during the follow-up over a period of 18 months. RESULTS: The examinations performed 1 week after the decompression sickness accident and the hyperbaric oxygen therapy showed diminution of foveal reflectivity associated with retinal pigment epithelium modifications, deficit of choroidal perfusion in the macular region, and important central scotoma in both eyes. During the follow-up, the central scotoma disappeared after 6 months. The retinal pigment epithelium presented pigmentary migrations without subretinal fluid accumulation. CONCLUSION: This case report describes choroidal ischemia resulting from decompression-induced intravascular gaseous microemboli caused by a decompression sickness diving accident.

Modifications in corneal biomechanics and intraocular pressure after deep sclerectomy.

PURPOSE: To compare preoperative and postoperative measurements of corneal biomechanical properties and intraocular pressure (IOP) using Goldmann applanation tonometry (GAT) and the ocular response analyzer (ORA) in eyes undergoing deep sclerectomy with collagen implant (DSCI). PATIENTS AND METHODS: Thirty eyes of 30 glaucomatous patients undergoing deep sclerectomy with collagen implant and 30 eyes of 30 normal subjects were included. Goldmann applanation IOP, central corneal thickness (CCT), and ORA measurements [corneal compensated IOP (IOPcc), Goldmann-correlated IOP (IOPg), corneal resistance factor (CRF), and corneal hysteresis (CH)] were taken the day before deep sclerectomy with collagen implant and on days 1, 8, and 30 after surgery. Preoperative CH values were correlated to the number of preoperative medications and to the CCT. Also, the ORA measurements of normal subjects were compared with the preoperative measurements of the glaucomatous patients. RESULTS: The 30 glaucomatous eyes showed a statistically significant difference between preoperative and day 1 postoperative GAT IOP, ORA IOPcc, IOPg, CRF, and CH measurements. No statistically significant difference was noted when comparing the GAT IOP, ORA IOPcc, IOPg, and CRF measurements between days 1, 8, and 30 after DSCI.On the contrary, CH values were statistically different when comparing days 1, 8, and 30 after DSCI. The 30 normal eyes showed no statistical difference when compared with the preoperative IOPg parameter in the 30 glaucomatous eyes; the mean CRF and the mean CH values were statistically significantly higher than the preoperative values of the glaucomatous group. CONCLUSIONS: CH statistically increased between preoperative and postoperative day 1 DSCI. On days 8 and 30, the change in CH values was statistically significant.

Long-term efficacy and safety of low-dose interferon alpha2a therapy in severe uveitis associated with Behçet disease.

PURPOSE: To investigate long-term responsiveness of patients with severe uveitis associated with Behçet disease to interferon alpha2a (IFN-alpha2a). DESIGN: Retrospective study. METHODS: Thirty-two patients with sight-threatening uveitis associated with Behçet disease who relapsed despite corticosteroids and immunosuppressive agents were included. IFN-alpha2a was administered subcutaneously (three million units thrice a week). Ophthalmologic examination and fluorescein angiography associated with laboratory tests were performed at regular intervals. Main outcome measures were visual acuity and recurrence of uveitis attacks before, during, and after initiation of IFN-alpha2a therapy. RESULTS: Control of inflammation was achieved in 28 of 32 patients (88%). Mean observation period of the 28 responder patients was 70.6 months (range, 30.3 to 129.2 months). Median visual acuity improved from 0.52 to 0.33 (logarithm of the minimum angle of resolution units; P = .005) two years after initiation of IFN-alpha2a therapy. The relapse rate decreased significantly during IFN-alpha2a treatment from 1.68 +/- 1.22 relapses/patient/year to 0.11 +/- 0.20 relapses/patient/year (P < .0001). IFN-alpha2a was discontinued in 19 of 28 patients (68%) after 32 months of treatment (range, 16 to 50 months). Mean follow-up after IFN-alpha2a discontinuation was 43 months (range, 11 to 84 months). After IFN-alpha2a discontinuation, the relapse rate increased from 0.08 +/- 0.21 relapse/person/year to 0.74 +/- 1.40 relapse/person/year (P = 0.04). CONCLUSIONS: IFN-alpha2a is efficient and safe for the long-term management of severe uveitis associated with Behçet disease. Meanwhile it seems to be a suspensive therapeutic strategy, even though long-term remission is possible in some patients.

Efficacy of interferon alpha in the treatment of refractory and sight threatening uveitis: a retrospective monocentric study of 45 patients.

AIM: Severe uveitis is potentially associated with visual impairment or blindness in young patients. Therapeutic strategies remain controversial. The efficacy of interferon alpha-2a (IFN-alpha2a) in severe uveitis, refractory to steroids and conventional immunosuppressive agents, was evaluated. PATIENTS AND METHODS: Patients were included after a major relapse of uveitis following corticosteroids and immunosuppressants. IFN-alpha2a (3 million units three times a week) was administered subcutaneously. Efficacy was assessed by improvement in visual acuity, decrease in vitreous haze, resolution of retinal vasculitis and macular oedema, assessed by fundus examination and fluorescein angiography, and decrease in oral prednisone threshold. RESULTS: 45 patients were included. Median age was 32.3 years (range 8-58) and sex ratio (F/M) was 0.66. Uveitis was associated with Behçet's disease in 23 cases (51.1%) and with other entities in 22 cases (48.9%). Median duration of uveitis before interferon therapy was 34.9 months (range 3.4-168.7) and an average of 3.26 relapses following corticosteroids and immunosuppressants was noted. Uveitis was controlled in 82.6% of patients with Behçet's disease and 59% of patients with other types of uveitis (p = 0.07). During a mean follow-up of 29.6 months (range 14-55), median oral prednisone threshold decreased significantly from 23.6 mg/day (range 16-45) to 10 mg/d (range 4-14) (p<0.001). Interferon was discontinued in 10 patients (22.2%) with Behçet's disease and in four patients without Behçet's disease. Relapses occurred in four and one cases, respectively. CONCLUSIONS: Interferon therapy appears to be an efficient strategy in severe and relapsing forms of Behçet's disease but also in other uveitic entities. However, it seems to act more to suspend rather than cure the disease. Therefore, IFN-alpha2a may be proposed as a secondline strategy after failure of conventional immunosuppressants.