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.

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.

A Novel Mutation of KIF11 in a Child with 22q11.2 Deletion Syndrome Associated with MCLMR.

Microcephaly with or without chorioretinopathy, lymphedema, or mental retardation (MCLMR; OMIM 152950) is a rare autosomal dominantly inherited syndrome. Mutations in the kinesin family member 11 (KIF11) gene have been associated with this condition. Here, we report a de novo novel heterozygous missense mutation in exon 12 of the KIF11 gene [c.1402T>G; p.(Leu468Val)] in a boy with 22q11.2 microdeletion syndrome. His major features were microcephaly, ventricular septal defect, congenital lymphedema of the feet, and distinct facial appearance including upslanting palpebral fissures, a broad nose with rounded tip, anteverted nares, long philtrum with a thin upper lip, pointed chin, and prominent ears. His right eye was enucleated due to subretinal hemorrhage and retinal detachment at age 3 months. Lacunae of chorioretinal atrophy and the pale optic disc were present in the left eye. He also had a de novo 1.6-Mb microdeletion in the Di George/VCFS region of chromosome 22q11.2 in SNP array, which was confirmed by FISH analysis. In this study, for the first time, we describe the co-occurrence of a KIF11 mutation and 22q11.2 deletion syndrome in a patient with MCLMR.

Intravitreal bevacizumab monotherapy for type-1 prethreshold, threshold, and aggressive posterior retinopathy of prematurity - 27 month follow-up results from Turkey.

PURPOSE: To study the efficacy of intravitreal bevacizumab (IVB) injection as a single treatment for retinopathy of prematurity (ROP). METHODS: This was a prospective interventional case series study performed in a clinical practice setting; a total of 122 patients including prethreshold (type 1) (n = 79, 152 eyes, six unilateral), threshold (n = 12, 24 eyes), and aggressive posterior (APROP) (n = 31, 62 eyes); cases were included without any randomization or masking. A total of 253 IVB injections, 238 in the first session, 11 in the second session, and four in the third session were performed, and followed up for a mean of 89.155 ± 4.277 (range 82 to 105) weeks of postmenstrual age (PMA). Regression of ROP, maturation of the retina, and associated complications were evaluated. RESULTS: Total regression was achieved in 227/238 eyes (95.4 %) after the first dose injection. The remaining 11 received a second injection, after which an additional seven (234/238; 98.2 %) regressed; after the third injection, the remaining 4 (238/238; 100 %) regressed. Complete retinal vascular maturation was achieved without any significant complications in all of the cases. CONCLUSIONS: IVB injection as monotherapy seems to be a very effective treatment modality for ROP. Based on timely intervention, IVB as a single treatment modality can salvage almost all ROP cases before stage 4.

Tenon advancement and duplication technique to prevent postoperative Ahmed valve tube exposure in patients with refractory glaucoma.

PURPOSE: To present and compare the long-term results of Dr. Tamcelik's previously described technique of Tenon advancement and duplication with the conventional Ahmed glaucoma valve (AGV) implantation technique in patients with refractory glaucoma. METHODS: This study was a multicenter, retrospective case series that included 303 eyes of 276 patients with refractory glaucoma who underwent glaucoma valve implantation surgery. The patients were divided into three groups according to the surgical technique applied and the outcomes compared. In group 1, 96 eyes of 86 patients underwent AGV implant surgery without patch graft; in group 2, 78 eyes of 72 patients underwent AGV implant surgery with donor scleral patch; in group 3, 129 eyes of 118 patients underwent Ahmed valve implant surgery with "combined short scleral tunnel with Tenon advancement and duplication technique". The endpoint assessed was tube exposure through the conjunctiva. RESULTS: In group 1, conjunctival tube exposure was seen in 11 eyes (12.9 %) after a mean 9.2 ± 3.7 years of follow-up. In group 2, conjunctival tube exposure was seen in six eyes (2.2 %) after a mean 8.9 ± 3.3 years of follow-up. In group 3, there was no conjunctival exposure after a mean 7.8 ± 2.8 years of follow-up. The difference between the groups was statistically significant. (P = 0.0001, Chi-square test). CONCLUSION: This novel surgical technique combining a short scleral tunnel with Tenon advancement and duplication was found to be effective and safe to prevent conjunctival tube exposure after AGV implantation surgery in patients with refractory glaucoma.

Scleral buckling with a non-contact wide-angle viewing system.

PURPOSE: To assess the outcome of scleral buckling surgery using a non-contact wide-angle viewing system for fundus visualization in patients with rhegmatogenous retinal detachment without proliferative vitreoretinopathy. PATIENTS AND METHODS: Sixteen eyes of 16 patients underwent scleral buckling using a non-contact wide-angle viewing system combined with a 25-gauge illumination fibre inserted into the sclera at the pars plana. RESULTS: The mean age of the patients was 53.6 ± 13.7 years and the mean follow-up time was 13.4 ± 2.8 months. Retinal reattachment was achieved in 13 of the 16 eyes (81%). Three eyes underwent vitrectomy with silicone oil injection because of development of proliferative vitreoretinopathy in 2 eyes and scleral perforation due to excessive indentation during cryoretinopexy in 1 eye. Two eyes developed limited subretinal haemorrhage during subretinal fluid drainage. CONCLUSION: Simultaneous use of a non-contact wide-angle viewing system combined with a 25-gauge light fibre illumination for fundus visualization brings the advantages of microsurgery and indirect ophthalmoscopy into scleral buckling surgery.

A novel surgical technique to prevent postoperative Ahmed valve tube exposure through conjunctiva: tenon advancement and duplication.

BACKGROUND AND OBJECTIVE: To present and evaluate a novel surgical technique to prevent exposure of the Ahmed valve implant tube (New World Medical, Rancho Cucamonga, CA) through conjunctiva in congenital glaucoma called "combined short scleral tunnel technique with Tenon advancement and duplication." PATIENTS AND METHODS: Patients with failed previous congenital glaucoma surgeries were divided into two groups. In group 1, 32 eyes of 28 patients with a mean age of 8.82 +/- 5.39 years underwent classic Ahmed valve implant surgery. In group 2, 28 eyes of 24 patients with a mean age of 8.79 +/- 5.55 years underwent the novel combined short scleral tunnel with Tenon advancement and duplication technique. RESULTS: After a mean 31.68 +/- 9.25 months of follow-up, conjunctival tube exposure was seen in 3 patients (9.4%) in group 1. There was no conjunctival tube exposure in group 2 after a mean 34.96 +/- 7.93 months of follow-up. CONCLUSION: The combined short scleral tunnel with Tenon advancement and duplication technique is able to prevent conjunctival tube exposure after Ahmed valve implantation surgery in patients with congenital glaucoma.

Association of conjunctival and corneal calcification with vascular calcification in dialysis patients.

BACKGROUND: Conjunctival and corneal calcification (CCC) is a well-known and easily detectable extraskeletal calcification, but its association with vascular calcification was not investigated previously. The aim of this study is to investigate the relationship of CCC with vascular calcification and bone metabolism parameters in dialysis patients. METHODS: We evaluated 63 patients (30 men, 33 women; mean age, 43.5 +/- 13.4 years) who were on dialysis therapy for more than 6 months. Forty-four patients were on peritoneal dialysis and 19 patients were on hemodialysis therapy. The same observer evaluated the presence of CCC by using a slit-lamp microscope, and a total CCC score was recorded for each patient. Fifty-two age- and sex-matched healthy controls also were evaluated by using the same method. Biochemical data were collected from patient files. Bone mineral density (BMD) of the lumbar spine and femoral neck was measured, and the presence of vascular calcification was assessed by using x-ray examinations of the pelvis and hands. RESULTS: Mean CCC score in patients was significantly higher than that in controls (6.2 +/- 5.1 versus 1.3 +/- 1.8; P = 0.001). CCC score correlated significantly with duration of renal replacement therapy ( r s = 0.392; P = 0.002), serum phosphorus level ( r s = 0.259; P = 0.042), and calcium x phosphorus product ( r s = 0.337; P = 0.007). However, we did not find a significant correlation with calcium, parathyroid hormone, alkaline phosphatase, albumin, or C-reactive protein level or BMD. The frequency of vascular calcification was significantly greater in patients with a high CCC score (CCC score > or = 10) compared with a low CCC score (< or =3; 56.3% versus 5.6%; P = 0.002). CONCLUSION: Evaluation of CCC score is an easy, fast, and noninvasive method. It seems that CCC score can be used as an additional tool to assess the status of extraskeletal calcification in dialysis patients.

Structural features of attached retina in rhegmatogenous retinal detachments.

PURPOSE: To examine the thickness and structural features of the ophthalmoscopically normal-appearing, attached retina in rhegmatogenous retinal detachments by optical coherence tomography (OCT) and compare them with those of the fellow eye. METHODS: Seventy eyes of 35 patients with rhegmatogenous retinal detachments were included in this study. The retinal detachments were not total. None of the patients had pathologic myopia or a history of ocular surgery. The fellow eyes were ophthalmoscopically normal and used as controls. Attached retinal sites of the eyes with a retinal detachment and the corresponding retinal sites of the fellow eyes were examined by OCT. Three OCT sections were taken from each eye, and thicknesses of three reflective zones corresponding to the inner part of the neurosensory retina (partial neurosensory retina), photoreceptor-photoreceptor outer segment, and total retinal thickness on each OCT section were measured and compared with those of the fellow eye. These three measurements were taken as the main outcome measures and performed on five different sites of each OCT section taken from each eye. RESULTS: The mean partial neurosensory retinal thickness of the attached retina in the eyes with a retinal detachment and the corresponding retinal sites in the fellow eyes was 172.74 +/- 14.23 microm and 170.60 +/- 16.51 microm, respectively. There was no statistically significant difference between these two values. The mean photoreceptor outer segment thickness of the attached retina in the eyes with a retinal detachment and the corresponding retinal sites in the fellow eyes was 62.74 +/- 12.02 microm and 58.23 +/- 15.14 microm, respectively. There was a statistically significant difference between these two values. The mean total retinal thickness of the still-attached retina in the eyes with a retinal detachment and the corresponding retinal sites in the fellow eyes were 293.23 +/- 27.87 microm and 277.06 +/- 19.12 microm, respectively. There was a statistically significant difference between these two values. CONCLUSIONS: The main difference between the two groups of eyes was at the level of the thickness of the black hyporeflective zone, corresponding anatomically to the photoreceptor and photoreceptor outer segment that enlarges in the retinal detachments. Therefore, there may be a subclinical retinal detachment or a global retinal relaxation even in the ophthalmoscopically normal-appearing, still-attached retina. This may be the underlying cause of preoperative and postoperative signs and symptoms. Additional studies are required to confirm these findings.

Early surgery for dysthyroid orbitomyopathy based on magnetic resonance imaging findings.

PURPOSE: To investigate the value of magnetic resonance imaging (MRI) in the determination of the fibrotic phase in dysthyroid orbitopathy, which may be an indication for early surgery. PATIENTS AND METHODS: Thirteen patients with dysthyroid orbitopathy who had vertical deviation in excess of 20 PD and associated diplopia in the primary position that did not respond to medical treatment were included in the study. On MRI, the absence of high-intensity foci in the T2 sequence, especially in the coronal planes, indicated that the extraocular muscles were not edematous. These muscles were evaluated as being in the fibrotic phase, and surgery was performed without waiting the recommended 6 months for the stabilization of the angle of deviation. RESULTS: The complaint of diplopia disappeared after a single operation in 8 patients, and after a second operation in 4. Thus, success was obtained in 12 of the 13 patients. One patient with persistent vertical diplopia refused a second operation. CONCLUSION: Our results indicate that MRI is an effective and reliable method for the determination of the fibrotic phase in patients with dysthyroid orbitopathy who have diplopia in the primary position, and that MRI results have an important role in determining whether early surgery is appropriate.

Determining the lowest trypan blue concentration that satisfactorily stains the anterior capsule.

PURPOSE: To determine the lowest concentration of trypan blue that will stain the anterior capsule satisfactorily to perform a safe continuous curvilinear capsulorhexis and to describe a staining technique using a dispersive viscoelastic material. SETTING: Cerrahpasa School of Medicine, University of Istanbul, Istanbul, Turkey. METHODS: Trypan blue 0.1% (Vision Blue) was diluted by half by adding an equal volume of balanced salt solution (BSS) in a stepwise pattern continuously until the concentration failed to stain the anterior capsule satisfactorily. After the dilution, 4 concentrations of the dye were obtained: 0.050%, 0.025%, 0.0125%, and 0.00625%. The volume of dye used for staining was 0.1 cc at all concentrations. Each concentration of the dye was applied using a classic air-bubble technique or a technique in which the dye was injected under sodium hyaluronate 3.0%-chondroitin sulfate 4.0% (Viscoat) onto the anterior lens surface without using an air bubble to reform the anterior chamber. Each diluted concentration was used in 10 eyes by the same surgeon, with 5 eyes having the air-bubble technique and 5 having the viscoelastic technique. The 0.1% concentration was used in 5 eyes, all having the viscoelastic technique. RESULTS: Trypan blue in concentrations as low as 0.0125% stained the anterior capsule satisfactorily. In addition, staining was possible under the Viscoat. The viscoelastic technique was faster, safer, and easier to perform than the air-bubble technique. CONCLUSIONS: As trypan blue is a potentially carcinogenic vital dye and its possible long-term side effects are unknown, the lowest effective concentration should be used. A concentration lower than 0.1% was effective in staining the anterior capsule even under dispersive viscoelastic material.