Long-Term Outcome of Descemet Membrane Endothelial Keratoplasty in Eyes With Fuchs Endothelial Corneal Dystrophy Versus Pseudophakic Bullous Keratopathy.

PURPOSE: The aim of this study was to compare the long-term outcome of Descemet membrane endothelial keratoplasty (DMEK) in patients with Fuchs endothelial corneal dystrophy (FECD) and pseudophakic bullous keratopathy (PBK). METHODS: Records of consecutive DMEK surgeries performed between 2015 and 2016 at the Department of Ophthalmology, Cologne, Germany, were retrospectively reviewed from the prospective Cologne DMEK Database. Eyes with either PBK or FECD with a complete 3-year follow-up were enrolled. Main outcome parameters included central corneal thickness (CCT), peripheral corneal thickness (PCT), best spectacle-corrected visual acuity (BSCVA, logarithm of the Minimum Angle of Resolution), and endothelial cell count (ECC) before and after DMEK. RESULTS: Four hundred two eyes from 402 patients were included (FECD n = 371, PBK n = 31). Preoperatively, CCT (FECD: 681.91 ± 146.78 µm; PBK: 932.25 ± 319.84 µm) and PCT (FECD: 732.26 ± 98.22 µm; PBK: 867.54 ± 88.72 µm) were significantly higher in the PBK group (P < 0.01). Three years after DMEK, CCT (FECD: 526.56 ± 27.94 µm; PBK 663.71 ± 132.36 µm) was significantly lower in both groups compared with the preoperative values (P < 0.01), whereas PCT showed no significant difference. PCT increased during the course in the PBK group from month 12 after DMEK (12 mo: 783.73 ± 127.73 µm; 24 mo: 837.50 ± 110.19 µm; 36 mo: 857.79 ± 140.76 µm). The increase in PCT correlated with an accelerated ECC loss starting 12 months after DMEK (P = 0.036). Before DMEK, BSCVA in FECD was significantly higher (P < 0.001) compared with that in PBK. After 3 years, BSCVA improved in FECD and PBK eyes without significant difference (P = 0.239). CONCLUSIONS: Visual acuity after DMEK in PBK and FECD seems to be comparable during the long-term follow-up. Peripheral and central corneal edema seems to recur faster in eyes with PBK than in those with FECD. Therefore, using a donor graft with higher ECC or possibly a larger graft could be a promising approach for PBK patients.

Dry anophthalmic socket syndrome - morphological alterations in meibomian glands.

PURPOSE: To evaluate morphological alterations of meibomian glands (MGs) in the dry anophthalmic socket syndrome (DASS). METHODS: Fifteen unilateral anophthalmic patients wearing cryolite glass prosthetic eyes were enrolled. All patients with clinical blepharitis or other significant eyelid abnormalities were excluded. In vivo laser scanning confocal microscopy (LSCM) of the MGs in the lower eyelids both on the anophthalmic side and the healthy fellow eye was performed to quantify acinar unit density, acinar unit diameter, acinar unit area, meibum secretion reflectivity, the inhomogeneous appearance of the glandular interstice, and inhomogeneous appearance of the acinar walls. RESULTS: The lower eyelids of the anophthalmic sockets revealed a significant reduction of the acinar unit density (p = 0.003) as well as a significantly more inhomogeneous appearance of the periglandular interstices (p = 0.018) and the acinar unit walls (p = 0.015) than the healthy fellow eyelid. However, there were no significant differences regarding the acinar unit diameter, acinar unit area, and meibum secretion reflectivity of the MGs on the anophthalmic side compared to the healthy fellow eyelid (p ≥ 0.05, respectively). CONCLUSIONS: The eyelids of anophthalmic sockets without clinical blepharitis demonstrate a reduced density of MG acinar units and a more inhomogeneous appearance of the periglandular interstices and the acinar unit walls. This can cause meibomian gland dysfunction contributing to DASS and suggests early treatment of these symptomatic patients, even in the clinical absence of any blepharitis signs.

Corneal densitometry: a potential indicator for early diagnosis of Fabry disease.

PURPOSE: To assess corneal densitometry in patients with Fabry disease (FD) and to compare corneal densitometry differences in FD patients to different corneal manifestations. METHODS: Ten participants (20 eyes) with FD and 10 age-matched healthy volunteers (20 eyes) were recruited. All participants were assessed by standardized ophthalmic examinations and the corneal densitometry analysis by Pentacam HR. Densitometry measurements were analyzed in standardized grayscale units. RESULTS: Seven patients developed conjunctival vessel tortuosity, cornea verticillata appeared in 6 patients, and two patients had Fabry cataract. Retinal vessel tortuosity occurred in 4 patients, and dilation of retinal vessels appeared in 3 patients, all symptoms occurred in both eyes. The first diagnosis of FD up to examination was 4.7 ± 3.23 years, and first ERT up to examination was 2.6 ± 2.27 years. The initial time to diagnosis was negatively related to the corneal densitometry value of the 0-2-mm (r = - 0.556, p = 0.011) and 2-6-mm (r = - 0.482, p = 0.032) zones in the posterior layer. FD group have significantly higher corneal densitometry in anterior 0-2-mm zone and 2-10-mm zone anterior and posterior layer than the control group (p ≤ 0.035, respectively). When divided into two groups by the existence of cornea verticillata, there was a statistically significant difference in the anterior layer, 6-10-mm zone (p = 0.031); in the central layer, 0-2 mm (p = 0.012), 2-6 mm (p = 0.001), 6-10 mm (p = 0.002), and total (p = 0.002); and in the posterior layer, 6-10 mm (p = 0.004) and total (p = 0.002). CONCLUSIONS: FD patients show higher corneal densitometry, and corneal densitometry may have potential for early diagnosis and reminding progress of FD.

Fibrillar Layer as a Marker for Areas of Pronounced Corneal Endothelial Cell Loss in Advanced Fuchs Endothelial Corneal Dystrophy.

PURPOSE: We sought to assess the correlation of corneal endothelial cell (CEC) density to alterations of collagen composition of Descemet membrane (DM) in advanced Fuchs endothelial corneal dystrophy (FECD) and to image such changes by slit-lamp biomicroscopy in vivo. DESIGN: Prospective, observational consecutive case series. METHODS: Fifty eyes (50 subjects) with advanced FECD were enrolled. After slit-lamp biomicroscopy and corneal Scheimpflug imaging, the Descemet endothelium complex (DEC) was retrieved during DM endothelial keratoplasty (DMEK) surgery. The expression of collagens I, III, and IV (COL I, COL III, and COL IV) and corresponding CEC density were analyzed by immunofluorescence flat mount-staining. Presence, diameter and surface area of collagen expression, and CEC density served as the main outcome measures. RESULTS: Immunofluorescence staining revealed central coherent collagen positive areas (mean surface area = 10 mm2 ± 6 mm2) corresponding to a fibrillar layer burying the guttae of DM in 84% (42/50) of DECs. CEC density overlying the fibrillar layer compared with the periphery was significantly reduced (-54.8%, P < .0001) with a steep decline of CEC density at its borders. Subgroup analysis revealed that the fibrillar layer may be imaged by slit-lamp biomicroscopy in vivo with significant positive correlation of mean maximum diameter detected by slit-lamp biomicroscopy (dSL max = 4.1 mm ± 0.9 mm) and by immunofluorescence staining (dIF max = 4.7 mm ± 1.1 mm; r = 0.76; P = .001). CONCLUSION: A fibrillar layer with a clear geographic pattern marks areas of pronounced loss of CEC density in advanced FECD eyes and may be imaged by slit-lamp biomicroscopy in vivo.

Effect of Anticoagulant Therapy on the Outcome of Descemet Membrane Endothelial Keratoplasty.

PURPOSE: To analyze the effect of anticoagulant therapy (ACT) for systemic diseases on the outcome of Descemet membrane endothelial keratoplasty (DMEK). METHODS: Consecutive eyes with Fuchs endothelial dystrophy that underwent DMEK between August 4, 2011, and July 15, 2016, were retrospectively analyzed. Data were obtained from the Cologne DMEK database at the University of Cologne, Germany. Best spectacle-corrected visual acuity (logMAR), endothelial cell density (at baseline and postoperatively up to 12 months), and rebubbling rates were compared between patients receiving ACT (ACT group) and those with no anticoagulant treatment (NCT group). RESULTS: In this study, 329 eyes of 329 patients were included (ACT group n = 97, NCT group n = 232; mean age 69.9 ± 9.1 years). Bleeding was more common in the ACT group (P < 0.001). Preoperative best spectacle-corrected visual acuity was 0.59 ± 0.44 and 0.48 ± 0.35 logMAR for the ACT and NCT groups, respectively, which improved to 0.13 ± 0.08 and 0.08 ± 0.16 logMAR, respectively, at 12 months postoperatively. No significant difference in endothelial cell density loss at 12 months was found between the groups (ACT group 36.2% ± 14.7%, NCT group 38.5% ± 15.1%; P = 0.467). Rebubbling rate was 19.6% in the ACT group and 28.9% in the NCT group (P = 0.08). CONCLUSIONS: Although ACT increases the risk for preoperative and intraoperative bleeding in DMEK, there seems to be no negative effect on DMEK outcome. Thus, it is not advisable to stop ACT for DMEK surgery.

More than loss of taste and smell: burning watering eyes in coronavirus disease 2019.

OBJECTIVES: To evaluate ocular symptoms in European non-hospitalized patients with severe acute respiratory syndrome-related coronavirus 2 (SARS-CoV-2) and to investigate associations with the demographic data as well as nasal and general physical symptoms. METHODS: In this prospective, observational study, 108 non-hospitalized patients with PCR-confirmed SARS-CoV-2 infection not requiring intensive care were asked about disease-associated ocular symptoms, demographic data, as well as general physical and nasal symptoms using a standardized questionnaire. Total ocular symptom score (TOSS) was evaluated during and, retrospectively, before development of coronavirus disease 2019 (COVID-19). Associations between TOSS and demographic data as well as general and nasal symptoms were evaluated. RESULTS: Seventy-five of the 108 COVID-19 patients (69.4%) had at least one ocular symptom during COVID-19. The most common symptoms included burning sensations in 39 (36.1%), epiphora in 37 (34.3%) and redness in 28 (25.9%), compatible with conjunctivitis. These symptoms occurred 1.96 ± 3.17 days after the beginning of COVID-19 and were mild. TOSS was significantly higher during COVID-19 (1.27 ± 1.85) than before the infection (0.33 ± 1.04; p < 0.001). There were no significant associations between TOSS and gender (ß coefficient -0.108; p 0.302), age (-0.024; p 0.816), rhinorrhoea (-0.127; p 0.353), nasal itching (-0.026; p 0.803), sneezing (0.099; p 0.470), nasal congestion (-0.012; p 0.930), cough (-0.079; p 0.450), headache (0.102; p 0.325), sore throat (0.208; p 0.052), or fever (0.094; p 0.361). CONCLUSIONS: Ocular involvement in European non-hospitalized individuals with COVID-19 seems to be highly underestimated. Overall, these ocular symptoms, including burning sensations, epiphora and redness, seem to be mild and to not need treatment.

[The role of ophthalmology in the COVID-19 pandemic]. / Die Rolle der Augenheilkunde in der COVID-19-Pandemie.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causing coronavirus disease 19 (COVID-19) has led to a worldwide pandemic. This pandemic presents a huge challenge for the healthcare system and also for ophthalmologists. Previous studies and case reports indicated that SARS-CoV­2 also infects the conjunctiva resulting in conjunctivitis. In addition, infectious virus particles in the tear fluid can be potential sources of infection; however, the detection of SARS-CoV­2 RNA in the tear fluid has rarely been successful. Although isolated conjunctival involvement is highly unlikely, at the current point in time of the COVID-19 pandemic, practically every patient examined by an ophthalmologist could be infected with SARS-CoV­2. Therefore, protective and hygiene measures should currently be consistently followed to minimize the risk of spreading the virus. Currently, there are no treatment recommendations for conjunctivitis associated with COVID-19. Tear substitutes might be helpful for symptom relief but there is no evidence for a topical antiviral therapy. In the future ophthalmologists could play a decisive role in the screening of maculopathies that might occur during COVID-19 treatment using chloroquine or hydroxychloroquine.

Olfactory and Gustatory Dysfunction in Coronavirus Disease 2019 (COVID-19).

Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has spread rapidly worldwide. Given scarce resources, nonlaboratory diagnostics are crucial. In this cross-sectional study, two-thirds of European patients with confirmed COVID-19 reported olfactory and gustatory dysfunction, indicating the significance of these symptoms in early diagnostics.

Dry anophthalmic socket syndrome - Standardized clinical evaluation of symptoms and signs.

OBJECTIVES: To assess symptoms and signs of the dry anophthalmic socket syndrome (DASS) in a standardized manner. METHODS: 87 unilateral anophthalmic patients were evaluated using the Ocular Surface Disease Index (OSDI), the 5-Item Dry Eye Questionnaire (DEQ-5), and a modified version of Symptom Assessment iN Dry Eye (SANDE) questionnaire separately for the anophthalmic socket and for the healthy fellow eye. Conjunctival inflammation was semi-quantitatively graded and Schirmer I test with topical anesthesia was performed bilaterally. The correlations between scores of the dry eye questionnaires and the results of the Schirmer tests, conjunctival inflammation, and demographic data were examined. RESULTS: Patients had significantly higher OSDI, DEQ-5 and SANDE scores at the anophthalmic side compared to the healthy eye (p ≤ 0.019, respectively). 63% of patients complained of anophthalmic socket dryness in at least one of the three questionnaires. Patients had higher inflammation (p < 0.001) and more tear volume in the Schirmer I test with topical anesthesia (p ≤ 0.024) on the anophthalmic side compared to the fellow eye. CONCLUSIONS: Most anophthalmic patients have significantly more subjective dryness complaints on their anophthalmic side compared to the healthy fellow eye, even in absence of tear deficiency and clinical blepharitis. Eye care practitioners should consider the diagnosis criteria and the definition for DASS proposed in this study, when counseling anophthalmic patients. However, research should be undertaken to investigate the role and the interactions of causative etiological causes for DASS. Furthermore, there is a high priority to establish a standardized examination protocol and to develop an evidence-based treatment algorithm for DASS.

[Personalized medicine in the treatment of periocular tumors : Targeted treatment and use of immune checkpoint inhibitors]. / Personalisierte Medizin in der Behandlung von periokulären Tumoren : Zielgerichtete Therapien und der Einsatz von Immun-Checkpoint-Inhibitoren.

The aim of this article is to provide an overview on the current state of personalized medicine in the systemic treatment of selected periocular tumors, such as basal cell carcinoma, Merkel cell carcinoma and conjunctival melanoma. This article therefore provides an extensive current literature review from PubMed including the current guidelines and standard operating procedures (SOP). As 90% of basal cell carcinomas have a pathologic activation of the sonic hedgehog pathway, vismodegib is a new treatment option for inoperable or metastatic basal cell carcinoma and for patients with Gorlin-Goltz syndrome. A novel approach of systemic medicine for the treatment of metastatic Merkel cell carcinoma is immunotherapy using the immune checkpoint inhibitor avelumab. Other personalized immunotherapies, such as the checkpoint inhibitors pembrolizumab and nivolumab, the tyrosine kinase inhibitor pazopanib and the role of the hedgehog pathway in Merkel cell carcinomas are the subject of current research and will certainly play an important role in future treatment. In the narrow sense personalized medicine has only come true for metastatic conjunctival melanomas: systemic treatment with BRAF, MEK and/or checkpoint inhibitors is initiated only when a BRAF mutation is detected in the tumor or metastatic tissue. Systemic immunotherapy with the checkpoint inhibitors pembrolizumab and nivolumab can also be used as a treatment option in metastatic conjunctival melanomas. In summary, personalized medicine is a subject of current research and provides many new targeted treatment options especially for periocular malignancies; however, it also involves many great challenges in the development and implementation of new techniques and therapies.

Morning Myopic Shift and Glare in Advanced Fuchs Endothelial Corneal Dystrophy.

PURPOSE: Impaired subjective morning visual acuity with improvement of symptoms during the day is pathognomonic for corneal endothelial dysfunction in advanced Fuchs endothelial corneal dystrophy (FECD). This study aimed to analyze the daily fluctuations of corneal thickness, refraction, and (glare) visual acuity in advanced FECD. DESIGN: Prospective cohort study. METHODS: Patients with advanced FECD (FECD group) and patients with normal cornea (control group) were enrolled. Routine clinical examination was performed using slit-lamp biomicroscopy, funduscopy, and macular optical coherence tomography. In addition, assessment using corneal Scheimpflug tomography (Pentacam), refraction, corrected distance visual acuity (CDVA), and glare CDVA was performed at 4 PM (afternoon) and the following day at 8 AM (morning). RESULTS: A total of 29 FECD eyes and 22 control eyes were included. Diurnal variations from afternoon to morning were Δ corneal thickness (apex) ± standard deviation (SD) 41.45 ± 34.1 µm (P < .001, FECD group) and 5.5 ± 6.72 µm (P = .001, control group); Δ spherical equivalent ± SD -0.64 ± 0.6 diopters (D) (P < .001, FECD group) and -0.01 ± 0.50 D (P = .461, control group); Δ total corneal refractive power ± SD 0.60 ± 0.83 D (P = .001, FECD group) and -0.01 ± 0.49 D (P = .602, control group), Δ CDVA ± SD 0.15 ± 0.18 logarithm of minimal angle of resolution (logMAR) (P < .001, FECD group) and 0.02 ± 0.04 logMAR (P = .174, control group), Δ CDVA glare ± SD 0.34 ± 0.25 logMAR (P < .001, FECD group) and 0.05 ± 0.11 logMAR (P = .106, control group). CONCLUSION: A morning myopic shift and increased glare paralleling increased corneal thickness may particularly contribute to subjective visual impairment in advanced FECD in the first hours after awaking. This should be taken into account during assessment and surgical decision-making in patients with FECD.

[Young male patient with unusual space-occupying lesion of the lower eyelid]. / Junger Patient mit ungewöhnlicher Raumforderung am Unterlid.

BACKGROUND: A space-occupying lesion of the eyelid in young adults is often a sign of an inflammation, a trauma or a benign neoplasm. The aim of this case report is to demonstrate a rare basal cell carcinoma, which presumably already arose in adolescence without further high-risk factors. METHODS: A 28-year-old male patient presented for a second opinion on a painless swelling of the right lower eyelid. According to the patient's history the swelling had been present since the age of 16 years and originally resembled a molluscum contagiosum. An ophthalmologist in private practice made the diagnosis of a suspected trichoepithelioma. The extended patient history revealed a blood coagulation disorder. The clinical ophthalmological examination revealed a nodular space-occupying lesion with a border wall and telangiectasia. The further ophthalmological examination was bilaterally inconspicuous. Due to the suspicion of a malignant process, an operation was promptly carried out using local anesthesia with complete tumor excision and pedicled flap plasty as well as a histopathological investigation to confirm the diagnosis. RESULTS: The histopathological investigation revealed underlying infiltrates of a basaloid tumor with bale-shaped trabecular growth, sometimes with peripheral palisading of the cells. The cells were predominantly monomorphic with isolated pleomorphic nuclei and sometimes enclosed mitoses. Immunohistochemically the cells were strongly positive for BerEP4 and negative for epithelial membrane antigen (EMA). The diagnosis of a nodular basal cell carcinoma of the right lower eyelid was made. All incision margins were free of tumor cells (R0 resection). In the dermatological screening no further manifestations were detected. CONCLUSION: Despite the occurrence of a space-occupying lesion of the eyelid in a young adult patient and also with no further risk factors, in addition to an inflammatory event and a benign tumor, a malignant disease, such as a basal cell carcinoma should also be taken into consideration. A tissue biopsy or complete excision with subsequent histological examination including an immunohistochemical analysis are essential for differentiation from other tumor entities.

[Intubation Systems in Lacrimal Drainage Surgery - a Current Overview]. / Intubationssysteme in der Tränenwegchirurgie ­ eine aktuelle Übersicht.

The development of microendoscopes in lacrimal drainage surgery has led to the introduction of novel surgical procedures that maintain transcanicular anatomy, including a variety of lacrimal drainage intubation systems. Depending on the location and extent of the lacrimal duct stenosis, bicanaliculoanular, monocanalicular, monocanaliculonasal, bicanalicular, and bicanaliculonasal intubation techniques and systems, as well as a combination of these, may be used. For isolated intubation of the puncta lacrimalia, perforated punctum plugs may be used, while pure monocanalicular intubation can be performed using a Mini-Monoka. The monocanaliculonasal intubation systems include the Monoka (Wide Collarette type), Monoka of Fayet (Crawford type), the self-threading Monoka (Ritleng type), the Masterka, and the LacriJet for the ophthalmic surgeon. The bicanaliculonasal intubation systems include various BIKA systems, the Ritleng intubation system, the Crawford intubation system, and the FCI Nunchaku. Indications for both monocanaliculonasal and bicanaliculonasal intubation systems are obstructions, stenoses or lacerations of all kinds, as well as obstructions and stenoses after opening or after a DCR. For a conjunctivodacryocystorhinostomy (CDCR), the classical Lester Jones Tube, Metaireau Tubes, and StopLoss Jones Tube may be used. Although the study situation is ambiguous and therefore lacrimal drainage intubation is not considered mandatory, most ophthalmic surgeons do not forego intubation - despite the higher costs and slightly more protracted surgery. The surgeons' selection of the intubation system depends on the chosen form of intubation, costs and personal preferences and experience.

Customizing a Cryolite Glass Prosthetic Eye.

In Germany, Austria, and Switzerland, over 90% of ocularists still manufacture customized prostheses using cryolite glass from Thuringia. The present manuscript demonstrates this long-forgotten technique in detail. This manuscript shows some major advantages of manufacturing prosthetic eyes using cryolite glass in comparison to poly(methyl methacrylate) (PMMA). These advantages include a lighter weight of the prosthesis, higher levels of patient satisfaction, and only one appointment necessary for the customized manufacturing. Potential risk of breakage seems not to be a critical disadvantage for glass prosthetic eye wearers. However, in some patients, manufacturing a well-fitting prosthetic eye is not possible or reasonable due to anophthalmic socket complications such as post nucleation socket syndrome, scarred fornices, or an orbital implant exposure. This article gives ophthalmologists a better insight into ocularistic care in order to improve the essential interprofessional collaboration between ocularists and ophthalmologists.