Erdheim-Chester disease with chorioretinal and orbital involvement: a case report / Doença de Erdheim-Chester com envolvimento coriorretiniano e orbitário: relato de caso

ABSTRACT A 42-year-old woman presented with bilateral proptosis, chemosis, leg pain, and vision loss. Orbital, chorioretinal, and multi-organ involvement of Erdheim-Chester disease, a rare non-Langerhans histiocytosis, with a negative BRAF mutation was diagnosed based on clinical, radiological, and pathological findings. Interferon-alpha-2a (IFNα-2a) was started, and her clinical condition improved. However, 4 months later, she had vision loss with a history of IFNα-2a cessation. The same therapy was administered, and her clinical condition improved. The Erdheim-Chester disease is a rare chronic histiocytic proliferative disease that requires a multidisciplinary approach and can be fatal if left untreated because of multisystemic involvements.

RESUMO Uma mulher de 42 anos apresentou proptose bi-lateral, quemose, dor nas pernas e perda de visão. Com base em achados clínicos, radiológicos e patológicos, foi diag-nosticada doença de Erdheim-Chester com acometimento orbitário, coriorretiniano e multiorgânico. Trata-se de uma rara histiocitose não Langerhans negativa para a mutação BRAF. Foi iniciado tratamento com interferon alfa-2a (IFNα-2a) e o quadro clínico melhorou. No entanto, quatro meses depois, a paciente apresentou perda visual após a cessação do IFNα-2a. A mesma terapia foi administrada novamente e sua condição clínica melhorou novamente. A doença de Erdheim-Chester é uma doença proliferativa histiocítica crônica rara que necessita de uma abordagem multidisciplinar e pode ser fatal se não tratada, devido a envolvimentos multissistêmicos.

Efficacy and safety of intracorneal allogenic ring segment implantation in keratoconus: 1-year results.

BACKGROUND: To evaluate the safety and efficacy of corneal allogenic intrastromal ring segments in the management of keratoconus patients. METHODS: The retrospective, nonrandomized, interventional case series consisted of 65 keratoconic eyes of 49 consecutive patients who had ring segment-shaped corneal allografts (KeraNatural®) implanted in intrastromal tunnels created using a femtosecond laser. The main outcome measures were uncorrected visual acuity (UCVA), corrected distant visual acuity (CDVA), refraction, keratometry, and pachymetry. Computed tomography scans of the corneal surfaces were also performed preoperatively as well as 3, 6 and 12 months postoperatively. RESULTS: Mean age was 29.5 ± 7.3 years (median 29, range: 20-52 years). The mean UCVA improved from 0.91 ± 0.50 logMAR preoperatively to 0.40 ± 0.24 logMAR postoperatively at 6 month follow-up (p < 0.01) and the mean CDVA improved from 0.87 ± 0.20 logMAR preoperatively to 0.27 ± 0.06 logMAR postoperatively (p < 0.01). The mean spherical equivalent improved from -8.82 ± 4.57 to -3.45 ± 4.81D (p < 0.01). Average Keratometry decreased from 49.23 ± 5.22 preoperatively to 45.63 ± 4.89 D postoperatively (p < 0.01). Mean anterior and posterior maximum elevation were also decreased significantly (p < 0.01). In one patient, dislocation of the graft towards the tunnel incision site and dehiscense at the tunnel entrance were observed in the first week of the operation. Yellow-white deposits were observed in the segment tunnels in five cases after 6 months. CONCLUSION: This study demonstrated that implantation of corneal allograft ring segments is a viable alternative treatment for keratoconus with safety and good visual results.

Erdheim-Chester disease with chorioretinal and orbital involvement: a case report.

A 42-year-old woman presented with bilateral proptosis, chemosis, leg pain, and vision loss. Orbital, chorioretinal, and multi-organ involvement of Erdheim-Chester disease, a rare non-Langerhans histiocytosis, with a negative BRAF mutation was diagnosed based on clinical, radiological, and pathological findings. Interferon-alpha-2a (IFNα-2a) was started, and her clinical condition improved. However, 4 months later, she had vision loss with a history of IFNα-2a cessation. The same therapy was administered, and her clinical condition improved. The Erdheim-Chester disease is a rare chronic histiocytic proliferative disease that requires a multidisciplinary approach and can be fatal if left untreated because of multisystemic involvements.

The Effect of Lower Eyelid Malposition Repair on Palpebral Fissure Size Changes.

Objectives: The aim of the study was to evaluate the effect of changes in the horizontal and vertical palpebral fissure dimensions on surgical success performed due to entropion and ectropion of the lower eyelid. Methods: The present research was conducted as a retrospective and interventional case series who had undergone involutional lower eyelid malposition repair with a lateral tarsal strip (LTS) alone, LTS with a medial spindle, and LTS with the advancement of the lower eyelid retractors. The subjects' medical records, including demographic and clinic characteristics, pre-operative assessment of horizontal eyelid laxity, and surgical outcomes, were reviewed. The distance between the pupillary light reflex and the lower-eyelid margin marginal reflex distance 2 (MRD-2) and the horizontal palpebral aperture (HPA) width were measured using the Image J program in the pre-operative and post-operative 6-month follow-up. The correlation between surgical success, changes in pre-operative and post-operative MRD-2, and HPA width was assessed by the Spearman rank correlation test. Results: A total of 66 eyelids of 48 patients were included in the study. This cohort comprised of 41 males (86.4%) and 7 females (13.6%), 18 of whom underwent bilateral surgery. The pre-operative mean MRD-2 was 7.13 ± 1.98 mm, and the post-operative 6-month mean MRD-2 was 6.21±1.19 mm (p<0.01). The mean post-operative HPA width was statistically significantly higher in comparison with the mean pre-operative HPA width (27.35±2.41, 26.89±2.39, p=0.02, respectively). There was no correlation between success rate and changes in horizontal and vertical palpebral fissure dimensions. Conclusion: LTS surgery is a method that turns the shortened HPA width to normal and enables the successful correction of the lower eyelid malpositions.

Effect of capsular tension ring on refractive results in cases with pseudoexfoliation syndrome.

PURPOSE: To investigate the effect of capsular tension rings (CTRs) on postoperative refractive results in patients with pseudoexfoliation (PSX) syndrome. MATERIALS AND METHODS: Sixty-nine patients with PSX syndrome who had uncomplicated cataract surgery between March 2016 and February 2019 were reviewed retrospectively. The patients were divided into two groups. The 35 patients in Group 1 received CTRs prior to intraocular lens implantation during cataract surgery, and the 34 patients in Group 2 had cataract surgery without CTRs. Significant zonular weakness, uncontrolled glaucoma, and ocular pathologies causing low visual acuity were excluded. The preoperative and postoperative keratometry and autorefraction measurements, preoperative expected refractive values, and absolute refractive error were recorded. RESULTS: The mean ages of patients were 73.54 ± 9.78 years in Group 1 and 72.23 ± 6.72 years in Group 2 (p = 0.521). There was no statistically significant difference between Group 1 and Group 2 in terms of expected preoperative refraction values (-0.52 ± 0.12,-0.56 ± 0.08, respectively, p = 0.118). There was a statistically significant difference in postoperative spherical equivalent values between Group 1 and Group 2 (-0.05 ± 0.97 and -0.92 ± 0.57, respectively, p < 0.01). A statistically significant difference was found between the two groups in terms of absolute refractive error (0.46 ± 0.74 in Group 1 and -0.34 ± 0.59 in Group 2 p < 0.01). CONCLUSION: CTR implantation causes hyperopic shift, which should be taken into consideration when calculating the lens power of intraocular lens in patients with PSX syndrome.

Graft survival and clinical outcomes of Descemet membrane endothelial keratoplasty: long-term results.

PURPOSE: The aim is to report long-term graft survival rates and clinical outcomes of Descemet membrane endothelial keratoplasty (DMEK). METHODS: In this study 150 eyes that underwent DMEK whether for Fuchs endothelial corneal dystrophy (FECD) or for bullous keratopathy (BK) surveilled for 7 years at 6 time points to evaluate graft survival rates and clinical outcomes of post-corneal transplantation. RESULTS: Overall, the estimated survival probability of 95% confidence interval at 7 years of post-DMEK was 0.58 (0.72-0.77), and the survival probabilities of eyes operated for FECD (0.53) were higher than eyes operated for BK (0.42) (log rank 26.87, [p = 0.197]). Post-transplant eyes with FECD achieved better visual acuity levels than eyes with BK (p = 0.006). Primary graft failure occurred in 11.3% eyes. Secondary graft failure rate was 9.3%, and allograft rejection rate was 4.7%. CONCLUSION: Although DMEK is effective and safe in long term, visual results and graft survival rates are better in cases with FECD.

The Effect of Secondary Intraocular Lens Implantation Time on Visual Prognosis in Aphakia Cases after Open Globe Injury.

PURPOSE: We investigated the effect of the time of secondary intraocular lens (IOL) implantation on final visual acuity and other causes affecting visual prognosis in cases left aphakic after open globe injury. METHODS: The study included 62 eyes of 62 patients left aphakic after the repair of open globe injury between 2012 and 2019. Demographic characteristics, trauma zone, ocular trauma score, type of injury, time of secondary IOL implantation, final best-corrected visual acuity (BCVA), and complications were recorded for each patient. RESULTS: The mean follow-up time of 62 patients was 25.05 ± 12.59 months. The preoperative BCVA was found to be 2.40 ± 0.86 logarithm of the minimum angle of resolution (logMAR), while the postoperative final BCVA was found to be 0.53 ± 0.70 logMAR (p < 0.01). The mean interval timing of secondary sulcus foldable IOL implantation was determined to be 3.79 ± 4.04 months. No correlation was observed between secondary IOL implantation time and final BCVA (r = 0.140, p = 0.319). Furthermore, when only pediatric patients were taken, an excellent positive correlation was found between the secondary IOL implantation time and final BCVA logMAR (r = 0.895, p < 0.01). Multiple linear regression on final BCVA with age, revealed a significant model explaining 48.0% of the variability with younger age and better final BCVA with as significant coefficients (p = 0.007). CONCLUSIONS: Although time interval between primary repair and secondary IOL implantation to correct aphakia does not effect final BCVA in adult patients, earlier surgery should be considered for amblyopia management in pediatric patients.

Predictive Factors for Corneal Scarring in Progressive Keratoconus Patients after Corneal Collagen Cross-linking.

Objectives: To evaluate predictive factors for corneal scar formation following corneal collagen cross-linking (CXL).Methods: The medical records of 295 progressive keratoconus patients who had undergone accelerated CXL were reviewed retrospectively in this comparative cohort study. Thirty-two eyes of 32 patients who still had a corneal scar in the first year (scar group) and 75 eyes of 75 patients without any scar (control group) were included. The patients' demographic characteristics, preoperative uncorrected distance visual acuity (UCVA) and corrected distance visual acuity (CDVA), slit-lamp biomicroscopy and corneal topographic parameters data were acquired from the patients' files. Multivariable logistic regression analysis and Receiver operating characteristic (ROC) curves were used for the purpose of evaluating predictive factors for corneal scar formation.Results: The UCVA and CDVA, which were determined to be similar between the groups during the preoperative period (P = .63, P = .71, respectively), improved postoperatively in both groups (P = .98, p = .10, respectively). The thinnest point of corneal thickness was statistically lower in the scar group (p = .03). In multivariable logistic regression analysis, preoperative keratometric astigmatism was a predictive value showing postoperative scar development (OR 11.81 95% CI 2.46-56.62, p = .002). Keratometric astigmatism had the highest sensitivity (90%), specificity (86.7%), and accuracy (95%) for scar formation at the best cut-off point of 5.61 D according to the ROC curve.Conclusions: A higher tendency for stromal haze development following CXL was determined in eyes with thinner corneas and higher keratometric astigmatism with the best cut-off value of 5.61 D.

Comparing the outcome of Muller's muscle conjunctival resection for mild/moderate versus severe involutional aponeurotic ptosis.

AIMS: To compare predictability of the outcomes of Muller's muscle conjunctiva resection (MMCR) in patients with severe versus mild/moderate involutional aponeurotic ptosis. METHODS: This was a retrospective case-control study. All cases were recruited into the groups in terms of preoperative marginal reflex distance of upper eyelid (MRD-1). Group 1 consisted of patients with mild to moderate blepharoptosis was defined as an MRD-1 equal to 1.1 to 3 mm and Group 2 consisted of patients with severe blepharoptosis defined as MRD-1 ⩽ 1 mm. All patients underwent MMCR based on the response to phenylephrine and the amount of ptosis. MRD-1 obtained both manually and with ImageJ analysis program preoperatively and on the postoperative sixth month was compared. Successful surgical outcome criteria were defined as MRD-1 ⩾ 2.5 mm and inter-eyelid symmetry ⩽ 1 mm. RESULTS: A total of 97 eyes of 83 participants met the inclusion criteria for this study (Group 1 n = 50, Group 2 n = 47). In terms of MRD-1 success rates were 88% in Group 1, and 70.2% in Group 2 (p = 0.03). Preoperative MRD-1 which was measured by ImageJ, and the amount of resected tissue in mm were the predictive data of surgical outcome by the logistic regression analysis (OR 6.45, 95% CI 1.82-22.78, p = 0.004, OR 2.47, 95% CI 1.05-5.80, p = 0.037, respectively). CONCLUSION: The surgical success of MMCR can be predicted via tissue resection length and preoperative MRD-1. Higher surgical success rates were obtained in cases with mild-moderate ptosis and clinically acceptable success rates were obtained in cases with severe ptosis.

2D Analysis of Gold Weight Implantation Surgery Results in Paralytic Lagophthalmos.

OBJECTIVES: Gold weight implantation in the upper eyelid is a frequently performed treatment for paralytic lagophthalmos to prevent corneal exposure. A margin reflex distance of -1 and -2 (MRD1, MRD2), the palpebral fissure height (PFH), and the vertical lagophthalmos (LV) are 1-dimensional (1D) measurements used in follow-up. Because the exposure area is 2-dimensional (2D), this study was designed to investigate the results using both 1D and 2D analysis. METHODS: Ten patients who underwent pretarsal suborbicularis oculi gold weight implantation were included in the study. Photographs were taken with a digital camera and the images were analyzed using ImageJ software (US National Institutes of Health, Bethesda, MD, USA). The lagophthalmos area (LA) and ocular surface area (OSA) were measured in 2D in addition to the MRD1, MRD2, PFH, LV. Preoperative and postoperative values were compared using the Wilcoxon signed-rank test. Associations between parameters were evaluated using Spearman's correlation analysis. RESULTS: The mean age of the patients (7 male, 3 female) was 39.6±16.4 years (range: 14-60 years). The mean implant weight was 1.46 g (0.8-1.6 g). There were significant reductions in the MRD1, MRD2, PFH, OSA, LV, and LA values after surgery (p<0.05). The weight of the gold implant had a strong correlation with the PFH, OSA, MRD1, and MRD2, but not the LV or LA, preoperatively. The OSA was strongly correlated with the MRD1, PFH, and the implant weight, but not the MRD2. The LA was strongly correlated with the LV, preoperatively. In the postoperative period, the OSA was strongly correlated with the PFH and the MRD2 but not the MRD1, while the LA was strongly correlated with the LV, MRD1, and the PFH. CONCLUSION: It is easy to obtain 2D measurements using digital image analysis software, and they proved to be accurate and correlated strongly with 1D measurements. The OSA and LA measurements were significantly lower following upper eyelid gold weight implantation. The PFH and LV were compatible with the OSA and LA, preoperatively.

Alterations in Corneal Biomechanical and Topographic Features After Accelerated Crosslinking: 1-Year Results.

OBJECTIVES: To determine the biomechanical and topographic alterations within the first year after accelerated crosslinking (CXL) treatment in patients with keratoconus. METHODS: In this prospective study, 52 eyes of 52 patients with progressive keratoconus underwent accelerated CXL were included. All patients had a detailed preoperative ophthalmologic examination, including slit-lamp evaluation, Goldmann tonometry, fundoscopy, topography by Scheimpflug imaging (Sirius), and corneal biomechanical evaluation with a biomechanical waveform analysis device (ORA). Alterations in visual acuity and topographic findings were evaluated before the treatment and at 12 months follow-up. Corneal biomechanical features were obtained before the treatment, and at 1st, 3rd, 6th and 12th months. RESULTS: Uncorrected-visual acuity and best-corrected visual acuity both statistically significantly improved at 12th month (p=0.001). There were no statistically significant differences in keratometry values, whereas maximum K (AKfront) and symmetry index front (SIfront) decreased significantly (p=0.015 and p=0.009, respectively). Corneal thinnest point and volume also decreased significantly at 12th month (p=0.001 for both). Goldmann-correlated intraocular pressure (IOPg) and corneal compensated IOP (IOPcc) values transiently increased in the first three months, while corneal hysteresis (CH) and the corneal resistance factor (CRF) transiently decreased, with the difference not statistically significant (p>0.05). However, central corneal thickness significantly decreased at the end of the 12th month (p=0.001). CONCLUSION: Accelerated CXL seems to be effective in stopping the progression of keratoconus. Our findings showed transient alterations in biomechanical features, which will end with the preoperative values at the end of the 12th month. Further studies are needed to demonstrate the changes in corneal biomechanics in vivo.

Complication of micro-bypass stenting: the anterior chamber displacement of a Xen implant / Complicação de stent micro-bypass: o deslocamento da câmara anterior de um implante de Xen

ABSTRACT Although minimally invasive glaucoma surgery using different types of implants is a promising strategy for treating glaucoma, potential long-term complications require further evaluation. Here, we report a case of the anterior chamber displacement of a Xen implant due to a maneuver aimed at correcting a dysfunctional and bent subconjunctival implant.

RESUMO Embora a cirurgia de glaucoma minimamente in vasiva, que usa diferentes tipos de implantes, seja uma estratégia promissora para o tratamento do glaucoma, as possíveis complicações a longo prazo exigem uma avaliação mais aprofundada. Aqui, relatamos um caso de deslocamento da câmara anterior de um implante Xen devido a uma manobra que visa corrigir um implante subconjuntival disfuncional e dobrado.

Complication of micro-bypass stenting: the anterior chamber displacement of a Xen implant.

Although minimally invasive glaucoma surgery using different types of implants is a promising strategy for treating glaucoma, potential long-term complications require further evaluation. Here, we report a case of the anterior chamber displacement of a Xen implant due to a maneuver aimed at correcting a dysfunctional and bent subconjunctival implant.

Comparison of outcomes of conventional transcanalicular laser dacryocystorhinostomy and modified transcanalicular laser dacryocystorhinostomy.

INTRODUCTION: Transcanalicular laser DCR (TL-DCR) approaches have been developed for the formation of a fistula between the nasal cavity and the lacrimal sac to ensure the continuity of the lacrimal drainage system over the years. However, the success rate of TL-DCR has varied widely. AIM: To evaluate and compare the success rates of conventional transcanalicular multidiode laser dacryocystorhinostomy and modified transcanalicular multidiode laser dacryocystorhinostomy. MATERIAL AND METHODS: Ninety-one eyes of 91 adult patients admitted with epiphora and diagnosed with chronic dacryocystitis were included in the study. The participants were divided into two groups. Group 1 consisted of 55 patients who were treated with conventional transcanalicular laser dacryocystorhinostomy. Group 2 consisted of 36 patients to whom the same surgical procedure was applied with the difference of nasal mucosa excision prior to laser osteotomy. The groups' intraoperative surgical ostium size, perioperative and postoperative complications, operative times and success rates were compared. RESULTS: The mean follow-up periods for each group were 8.88 ±2.99 months and 10.28 ±4.47 months, respectively (p = 0.077). Intraoperative mean surgical ostium sizes were 31.85 ±14.98 mm2 and 42.25 ±18.09 mm2, respectively (p = 0.004). The mean operation time in group 1 was significantly shorter compared to group 2 (18.55 ±4.05 min and 24.44 ±3.18 min, respectively, p = 0.0001). The overall success rate was 65.45% in group 1 and 75.00% in group 2 (p = 0.335). CONCLUSIONS: Although the surgical ostium area was significantly greater in group 2, there was no significant difference in surgical success and patient satisfaction rates between the two groups.

Protective effect of pomegranate juice on retinal oxidative stress in streptozotocin-induced diabetic rats.

AIM: To investigate the effect of pomegranate juice (PJ) intake on overall oxidation status in retinas of diabetic rats. METHODS: Twenty-seven rats were divided into four groups as control (CO), diabetic (DM), control treated with PJ (CO-PJ), and diabetic treated with PJ (DM-PJ).The retina tissues were used to determine 8-hydroxy-2'-deoxyguanosine (8OHdG), malondialdehyde (MDA), reduced glutathione (GSH) levels, and the enzyme activities of superoxide dismutase (SOD) and glutathione peroxidase (GSH-Px). RESULTS: The levels of 8OHdG and MDA were significantly increased in the retina of DM group compared to CO group (P=0.001, P<0.001 respectively). Both 8OHdG and MDA levels were decreased in PJ-DM group compared to DM group (P=0.004, P<0.001 respectively). The activities of antioxidant enzymes GSH, SOD, and GDH-Px were significantly decreased in the retina of DM group compared to CO group (P≤0.01). GSH and GSH-Px activities were higher in PJ-DM group compared with DM group (P=0.010, P=0.042, respectively) but SOD activity was not statistically different (P=0.938). CONCLUSION: PJ intake is found to be effective in decreasing oxidative end products, and in increasing the activities of antioxidant enzymes in diabetic retinas of rats, which suggests it may be effective against oxidative stress in diabetic retinas.

Intraocular pressure measurement over soft contact lens by rebound tonometer: a comparative study.

AIM: To evaluate the intraocular pressure (IOP) measurements by Icare rebound tonometer over a contact lens in comparison with Goldmann applanation tonometry (GAT). METHODS: Fifty patients using contact lens were included in this study. One of the eyes of the patients was selected randomly and their IOP were measured by rebound tonometer with and without contact lens (RTCL, RT respectively) and by GAT, as well as their central corneal thickness (CCT) by optical pachymeter. The results of both methods were compared by correlation analysis, general linear method repeated measure and Bland-Altman analysis. RESULTS: Mean IOP values measured by RTCL, RT and GAT were 15.68±3.7, 14.50±3.4 and 14.16±2.8 (P<0.001), respectively. Mean IOP by RTCL was significantly higher than the measurements implemented by RT and GAT (P<0.001), while there was no difference between the measurements by GAT and RT (P=0.629). There was a good level of positive correlation between GAT and RTCL as well as RT (r=0.786 P<0.001, r=0.833 P<0.001, respectively). We have observed that CCT increase did not show any correlation with the differences of the measurements between RTCL and RT (P=0.329), RTCL and GAT (P=0.07) as well as RT and GAT (P=0.189) in linear regression model. CONCLUSION: The average of the measurements over contact lens by rebound tonometer was found to be higher than what was measured by GAT. Although this difference is statistically significant, it may be clinically negligible in the normal population.

Chronic Meningitis Complicating Intracranial Hypertension in Neurobrucellosis: A Case Report.

In neurobrucellosis, even though meningitis is encountered frequently, chronic intracranial hypertension is a rare manifestation. Early diagnosis and treatment is very important for the prevention of permanent visual loss secondary to poststasis optic atrophy in these cases. We report a case that presented with permanent visual loss secondary to intracranial hypertension in neurobrucellosis. Our goal is to draw attention to the consideration of neurobrucellosis in cases with papilla stasis, even in the absence of neurological findings in endemic areas.

Effects of levodopa on retina in Parkinson disease.

PURPOSE: To evaluate the effects of levodopa on retina, we assessed retina with optical coherence tomography (OCT) in Parkinson disease (PD). METHODS: Thirty-five patients with PD (17 with levodopa monotherapy, 18 untreated) and 11 healthy controls were included in this cross-sectional study. Unified Parkinson's Disease Rating Scale (UPDRS) and Hoehn & Yahr (H&Y) staging scale were used for the evaluation of disease severity. All retinal scans were performed using OCT. RESULTS: Total UPDRS and motor subscores were lower in untreated patients compared to patients with treated PD (p = 0.013, p = 0.033, respectively). There was no significant difference in the range of H&Y stages between the untreated and treated PD groups (p = 0.342). The average retinal nerve fiber layer thickness of the untreated (106.76 ± 10.55 µm) and treated (104.62 ± 8.23 µm) patients with PD were significantly thinner than those of controls (115.60 ± 9.11 µm) (p<0.005). However, there was no significant difference between the untreated and patients with treated PD (p = 0.780). No significant difference in mean values of average thickness of ganglion cell complex layer was observed among controls, patients with untreated PD, and patients with treated PD (p = 0.304). CONCLUSIONS: Although the disease in patients with treated PD was more severe than in the untreated group, no significant difference in the thickness of retina was found between the 2 groups. Therefore, we thought that levodopa might have a protective effect on retina in patients with PD.