Serum Lipoprotein(a) Is Not Associated with Graves' Ophthalmopathy.

Aim: To investigate the relationship of serum lipoprotein(a) [Lp(a)] and other serum lipids with presence of Graves' ophthalmopathy (GO). Methods: A total of 99 consecutive patients diagnosed with Graves' disease (GD), aged 18-65 years, who had not received prior treatment for GO, thyroid surgery, or radioactive iodine therapy, were recruited between June 2020 and July 2022. In addition, 56 healthy controls (HCs) were included as the control group. All patients underwent an ophthalmological examination, and were classified based on the presence of GO into the GO group (n = 45) and no GO group (n = 54). Fasting blood samples were collected from all participants to analyze serum lipid parameters, including Lp(a), total cholesterol, low-density lipoprotein (LDL) cholesterol, high-density lipoprotein (HDL) cholesterol, and triglycerides. Results: The median serum levels of Lp(a) were 5.7 [4.3-9.2] in the GO group, 6.7 [3.7-9.9] in the no GO group, and 4.7 [3-7.6] in the HC group. The intergroup comparisons of serum Lp(a) levels showed no significant result. The serum levels of total cholesterol, LDL cholesterol, HDL cholesterol, and triglycerides were also similar between the groups (P > 0.05 for all). However, when analyzing only euthyroid GD patients and the control group, the serum LDL cholesterol levels were found to be significantly higher in the euthyroid GO group [median: 132 interquartile range (IQR) (110-148) mg/dL] than in the HCs [median: 96 IQR (94-118) mg/dL] (P = 0.002). Conclusion: The findings of our study did not support the association between serum Lp(a) levels and GO.

Conjunctival leiomyosarcoma in a patient with xeroderma pigmentosum: 5-year follow-up without recurrence / Leiomiossarcoma da conjuntiva em paciente com xeroderma pigmentoso: acompanhamento de 5 anos sem recidivas

ABSTRACT Conjunctival leiomyosarcoma is a very rare soft tissue malignancy. Herein, we describe a conjunctival leiomyosarcoma case in a patient with another rare disease, xeroderma pigmentosum. The 27-year-old single-eyed xeroderma pigmentosum patient complained of exophytic mass covering the ocular surface in her left eye. A vascular, hemorrhagic mass covering the entire ocular surface of the left eye was identified on the examination. Thus, total mass excision surgery was performed. The pathological diagnosis was compatible with conjunctival leiomyosarcoma. Additional chemotherapy, radiotherapy, or surgery were not accepted by the patient. No recurrence or metastasis was observed during the 5-year follow-up. Both primary conjunctival leiomyosarcoma and xeroderma pigmentosum are very rare diseases. Conjunctival masses in xeroderma pigmentosum patients should be approached carefully, and histopathological examination is warranted. For conjunctival leiomyosarcoma, early diagnosis, localized, unspread disease, and complete resection provide the best prognosis.

RESUMO O leiomiossarcoma da conjuntiva é um tumor ma­ligno de tecidos moles muito raro. Aqui é descrito um caso de leiomiossarcoma da conjuntiva em um paciente com xeroderma pigmentoso, que também é uma doença rara. Um paciente de 27 anos de idade com xeroderma pigmentoso de olho único queixou-se de uma massa exofítica cobrindo a superfície ocular do olho esquerdo. Ao exame, foi observada uma massa vascular hemorrágica cobrindo toda a superfície ocular do olho esquerdo. Foi realizada uma cirurgia de excisão total dessa massa. O diagnóstico patológico foi compatível com leiomiossarcoma da conjuntiva. O paciente recusou qualquer quimioterapia, radioterapia ou cirurgia adicional. Nenhuma recidiva ou metástase foi observada durante o acompanhamento de 5 anos. Tanto o leiomiossarcoma primário da conjuntiva quanto o xeroderma pigmentoso são doenças muito raras. Massas conjuntivais em pacientes com xeroderma pigmentoso devem ser abordadas com cuidado e deve-se realizar um exame histopatológico. Para o leiomiossarcoma conjuntival, o diagnóstico precoce, uma doença localizada e não disseminada e a ressecção completa proporcionam o melhor prognóstico.

Penetrating Keratoplasty in Congenital Glaucoma.

BACKGROUND: Childhood glaucoma is one of the most common causes of corneal opacity in childhood and is associated with various pathological corneal changes, including corneal enlargement, corneal clouding, and edema. Congenital glaucoma (CG) may cause a decrease in vision outcomes due to corneal opacity or clouding, which is often associated with stimulus deprivation amblyopia. Therefore, to create a balance between preventing amblyopia and sustaining corneal clearance, patients with CG can be managed with early penetrating corneal transplantation surgery along with advanced glaucoma management. AIM: To investigate the graft survival rate and factors affecting graft survival in patients with congenital glaucoma who underwent penetrating keratoplasty (PKP). STUDY DESIGN: Cross-sectional. MATERIALS AND METHODS: Patients with congenital glaucoma who underwent PKP were retrospectively evaluated. The associations between age, corneal diameter, presence of ocular comorbidities, concurrent ocular surgeries with corneal graft, and visual outcomes were assessed. RESULTS: Among the 30 eyes enrolled in the study, 6 (20%) had aniridia, 6 (20%) had Axenfeld-Rieger syndrome, and 18 (60%) were diagnosed with primary congenital glaucoma. Graft survival rates were 66.6% and 63.33% at 12 and 24 months, respectively. At the end of the follow-up, the overall graft survival rate was 60%. Statistical significance was observed between patient age at the time of surgery and graft failure (p = 0.02). Graft failure was associated with a younger patient age. Functional vision was achieved in 53.3% of patients. CONCLUSIONS: The management of congenital glaucoma and its corneal complications is a delicate issue that requires great effort. PKP in congenital glaucoma was moderately successful in the present study. To provide functional vision, PKP could be the treatment of choice.

Conjunctival leiomyosarcoma in a patient with xeroderma pigmentosum: 5-year follow-up without recurrence.

Conjunctival leiomyosarcoma is a very rare soft tissue malignancy. Herein, we describe a conjunctival leiomyosarcoma case in a patient with another rare disease, xeroderma pigmentosum. The 27-year-old single-eyed xeroderma pigmentosum patient complained of exophytic mass covering the ocular surface in her left eye. A vascular, hemorrhagic mass covering the entire ocular surface of the left eye was identified on the examination. Thus, total mass excision surgery was performed. The pathological diagnosis was compatible with conjunctival leiomyosarcoma. Additional chemotherapy, radiotherapy, or surgery were not accepted by the patient. No recurrence or metastasis was observed during the 5-year follow-up. Both primary conjunctival leiomyosarcoma and xeroderma pigmentosum are very rare diseases. Conjunctival masses in xeroderma pigmentosum patients should be approached carefully, and histopathological examination is warranted. For conjunctival leiomyosarcoma, early diagnosis, localized, unspread disease, and complete resection provide the best prognosis.

Retinal Vascular Patterns and Capillary Plexus Reflectivity of Intraocular Tumors; an Optical Coherence Tomography Angiography Study.

PURPOSE: To define the vascular characteristics of malignant and benign intraocular tumors by optical coherence tomography angiography (OCTA) and consequently to determine the advantages and disadvantages of the imaging technique in clinical practice. METHODS: This prospective, descriptive study consisted of choroidal hemangioma, choroidal nevus, choroidal melanoma, ocular melanocytoma, congenital hypertrophy of retinal pigment epithelium (CHRPE), and choroidal osteoma. Retinal angiography images were taken by OCTA in a 6 × 6 mm HD scan protocol. OCTA characteristics were defined by considering different tumor types separately. RESULTS: This study included 93 eyes of 90 patients diagnosed with benign or malignant intraocular tumors. The mean age of the patients was 48.9 ± 16.9 years in the hemangioma group, 55.3 ± 12.9 years in the nevus group, 48.2 ± 13.4 years in the melanoma group, 48 ± 18.9 years in the melanocytoma group, 45 ± 22.9 years in the CHRPE group, and 27.8 ± 7.8 years in the osteoma group. We showed four vascular patterns at the level of the choriocapillaris layer in circumscribed choroidal hemangiomas - the appearance of a "bag of worms," club-like appearance, giant choroidal vessels and normal choriocapillaris. The rates of these vascular patterns were 40%, 30%, 10%, and 20% in treatment-naïve hemangiomas and were 46.1%, 30.8%, 7.7%, and 15.4% in photodynamic therapy-treated hemangiomas, respectively. There was no different vascular structure in the tumor associated with the lesion in the nevus group. There were three different patterns at the choriocapillaris level in the melanomas - a vascular network (10%), avascular areas (30%) and vascular loops (60%). There were some atypical and nonspecific vascular changes demonstrated in the CHRPE and osteoma groups and a fine vascular network was observed in the melanocytoma group. CONCLUSION: The different types of intraocular tumors had specific vascular characteristics which were easily demonstrated by the OCTA machine. This imaging technique can be a useful tool to differentiate these intraocular tumors non-invasively.

Orbital compartment syndrome secondary to retrobulbar hematoma after infratrochlear nerve block for nasolacrimal probing.

After infratrochlear nerve block for nasolacrimal probing, sudden vision loss, proptosis, pain, loss of light reflexes, and a total limitation of ocular movement was observed in a 71-year-old female patient. She was diagnosed with retrobulbar hemorrhage and orbital compartment syndrome (OCS). Lateral canthotomy, cantholysis, and medial orbitotomy were performed on the patient. She was not taking any oral anticoagulant medication and did not have any disease other than hypothyroidism and systemic hy-pertension. All the clinical findings returned to normal right after the intervention except mild ptosis (~1 mm), which persisted for 2 months. All patients scheduled for periocular anesthesia should be questioned about using oral anticoagulant medications, and the possibility of serious complications should be kept in mind even for patients without any risk factors. Patients with OCS secondary to retrobulbar hemorrhage should be surgically managed within the critical window (90 min) to prevent any irrevers-ible optic nerve injury.

Corneal Endothelial Changes in Patients With Primary Congenital Glaucoma.

PRCIS: Specular microscopic parameters were found to be decreased in patients with primary congenital glaucoma (PCG). Patients with PCG and Haab striae had lower endothelial cell density (ECD) and central corneal thickness (CCT) than those without Haab striae did. The type of surgery (viscogoniotomy, viscotrabeculotomy, or combined viscotrabeculotomy and trabeculectomy) did not affect specular microscopic parameters. PURPOSE: The purpose of this study was to compare specular microscopic parameters such as corneal ECD, coefficient of variation (CV), hexagonal cell percentage (HEX), and CCT between patients with PCG and healthy controls; to determine the predictive ability of Haab striae for endothelial cell changes PCG by comparing patients with and without Haab striae; and to investigate if the type of surgery used to treat glaucoma (viscogoniotomy, viscotrabeculotomy, or combined viscotrabeculotomy and trabeculectomy) affects specular microscopic parameters. METHODS: A cross-sectional specular microscopic analysis of patients with PCG and healthy controls was performed. One hundred eyes of 62 patients with PCG with and without Haab striae and 101 eyes of 101 healthy controls were enrolled in this study. Specular microscopic parameters, including ECD, HEX, CV, and CCT, of all subjects were evaluated. RESULTS: Patients with PCG showed significantly lower ECD and CCT than healthy controls did. Those with Haab striae had lower ECD and CCT but no differences in age, HEX, and CV compared with those without Haab striae. ECD, CV, HEX, or CCT did not significantly differ among patients treated with the 3 different types of surgery. CONCLUSIONS: Prolonged exposure to elevated intraocular pressure during prenatal and/or early postnatal life results in structural changes in immature infant corneas. Specular microscopic parameters differed significantly between healthy controls and patients with PCG. The type of surgery had no effect on these parameters. Among patients with PCG, those with Haab striae had isolated corneal endothelial cell loss without morphologic changes within the endothelium.

Comparison of iodine-125 plaque brachytherapy and gamma knife stereotactic radiosurgery treatment outcomes for uveal melanoma patients.

PURPOSE: To compare the efficacies of iodine-125 brachytherapy (IBT) and gamma knife stereotactic radiosurgery (GKRS) in the treatment of posterior uveal melanoma. METHODS: The demographic data and tumor characteristics at diagnosis of 201 patients treated with IBT and 52 patients treated with GKRS were recorded. The two treatments were then compared in terms of complications, local control, eye retention, metastasis, and overall survival rate. RESULTS: The median follow-up time was 56 months for the GKRS group and 45 months for the IBT group (p = 0.167). There were no significant differences in demographic data or tumor characteristics between the groups at diagnosis. Radiation retinopathy, radiation optic neuropathy, and neovascular glaucoma occurred at similar rates in both groups. However, radiation maculopathy and cataracts occurred more frequently in the GKRS group. The number of cases that have developed vision loss (worsening of best-corrected visual acuity on three or more lines on the Snellen chart) was significantly higher in the GKRS group (60%) compared to the IBT group (44%) (p = 0.048). Local control, metastasis, and 5-year overall survival rates were statistically similar in both groups. CONCLUSIONS: GKRS can be preferred as an eye-sparing treatment option for posterior uveal melanoma in cases where brachytherapy cannot be used.

Does exogenous androgen use affect the retinal blood flow: An OCTA study in transgender men.

PURPOSE: This study aims to investigate the effect of using exogenous androgen on retinal microvascular structures in transgender men. METHODS: Retinal microvascular structures of transgender men and healthy women in the control group were visualized with optical coherence tomography angiography (OCTA). Foveal avascular zone (FAZ), superficial capillary vascular density (sCVD), deep capillary vascular density (dCVD), and radial peripapillary capillary (RPC) vascular density were evaluated. RESULTS: Thirty transgender men and 30 healthy women were included in the study. There were no significant differences between transgender men and the control group in terms of FAZ, central macular thickness, parafoveal, and perifoveal retinal thicknesses (p = 0.859, 0.288, 0.561, and 0.719; respectively). sCVD were found to be low in transgender men in the whole image, parafoveal, and perifoveal zones (p = 0.006, 0.025, and 0.005; respectively). Although there were low values of dCVD of the whole image, parafoveal, and perifoveal zones in transgender men, the difference was not statistically significant (p = 0.295, 0.085, and 0.270; respectively). RPC vessel densities in the whole image, peripapillary zone, and the inferior of the optic disc were found to be significantly low in transgender men (p = 0.003, 0.005, and 0.003; respectively). CONCLUSION: In this study, a decrease in vessel density in the superficial retinal layers and around the optic disc was detected in transgender men. These findings suggest that routine ophthalmological examination may be important in individuals who use exogenous androgens.

Investigation of Retinal Vascular Changes during Pregnancy Using Optical Coherence Tomography Angiography.

Purpose: To investigate retinal vascular changes in pregnant women using optical coherence tomography angiography (OCTA).Materials and Methods: This prospective, cross-sectional study included 97 pregnant women and age-matched 34 healthy control subjects. The OCTA parameters of one eye (left) of pregnancy group and control group were compared. Besides, the OCTA parameters of three trimesters were compared between themselves and the control group. The foveal avascular zone (FAZ) area and the vessel density percentages of the superficial capillary plexus (SCP), deep capillary plexus (DCP), and radial peripapillary capillary (RPC) of all the groups were evaluated by OCTA.Results: In the pregnancy group, the vessel density of DCP was higher in all regions than the control group except the foveal region, but this difference could not reach statistical significance. On the other hand, the vessel density of SCP in the pregnancy group did not show a manifest change in all regions except the foveal region as in DCP. Although the FAZ area was wider in pregnancy group, this difference was not statistically significant (p:0.661). There was no statistically significant difference observed in vessel density of SCP, DCP, and RPC between the different trimesters and the control group, and none of these data correlated with the gestational week.Conclusion: This study showed retinal vascular changes secondary to pregnancy, especially in the vessel density of DCP. These results can provide information that should be taken into account in the evaluation and investigation of pregnancy-specific retinal vascular pathologies.

Evaluation of the reasons for the microvascular changes in patients with Fabry disease using optic coherence tomography angiography.

PURPOSE: To investigate the blood flow changes in the choriocapillaris and the superficial and deep capillary plexus of the retina using optic coherence tomography angiography (OCTA) in patients with Fabry disease (FD) and reveal any possible association of these changes with the systemic findings. METHODS: This cross-sectional study included 38 patients with FD and age- and gender-matched 40 healthy controls. OCTA images were obtained from all patients. Superficial (sCVD) and deep capillary vascular density (dCVD) in the foveal, parafoveal, and perifoveal zones and the whole image were recorded for each patient. Flow area in the choriocapillaris and central macular thickness (CMT) were also recorded. RESULTS: Patients with FD showed a lower whole image (54.45 ± 5.99% vs 57.32 ± 6.71%, p = 0.004), foveal (34.94 ± 7.60% vs 39.65 ± 7.03%, p = 0.003), parafoveal (57.41 ± 4.85% vs 59.19 ± 4.67%, p = 0.043), and perifoveal (55.87 ± 6.43% vs 58.87 ± 7.02%, p = 0.003) dCVD compared to the healthy controls without a significant difference in the sCVD and choriocapillaris blood flow (p > 0.05). A significantly lower whole image and foveal dCVD in the FD patients with renal involvement was observed compared to the healthy controls (p = 0.027 and p = 0.024, respectively) without any significant difference between the FD patients without renal involvement and healthy controls (p = 0.17 and p = 0.13, respectively). CMT was significantly higher in FD patients with renal involvement compared to the ones without renal involvement (252.1 ± 18.5 µm vs 235.5 ± 17.6 µm, p = 0.016). CONCLUSION: Patients with FD showed a lower dCVD without any change in sCVD and choriocapillaris compared to the healthy controls. This decrease was associated mostly with the renal involvement and duration of treatment.

Investigation of clinical profile of Behçet's syndrome-related versus idiopathic branch retinal vein occlusion.

PURPOSE: To compare the long-term results of the patients with branch retinal vein occlusion (BRVO) secondary to Behçet's syndrome (BS) with the patients with unknown etiology. METHODS: Medical records and optical coherence tomography (OCT) imaging results of the patients with BRVO secondary to BS and with unknown etiology were reviewed retrospectively between 2016 and 2018 at a single center. The anatomical location of the BRVO, involvement of the macula, application of laser photocoagulation, and intravitreal injection were evaluated. RESULTS: Twenty-eight eyes of 23 patients with BRVO secondary to BS as the study group and 22 eyes of 19 idiopathic BRVO patients as the control group were included in the study. The mean duration of follow-up after the development of BRVO was 74.6 ± 57.4 months in the study group and 63.6 ± 59 months in the control group. The rate of bilaterality, macular involvement, and application of laser photocoagulation was not statistically significantly different between the groups. However, the frequency of injection requirement was significantly lower in the patients with BRVO secondary to BS in comparison to the control group (P= 0.009). CONCLUSION: Although the treatment of BRVO is laser photocoagulation and intravitreal injection of anti-VEGF agents or dexamethasone implant, the patients with BS might respond very well to systemic immunomodulatory agents in case of BRVO. Thus, rearrangement of the immunomodulatory treatment before starting intravitreal injections should be considered in the patients with BRVO secondary to BS.