Primary leiomyosarcoma of ovary: A rare malignancy as an incidental finding

Primary leiomyosarcoma (PLMS) of the ovary is extremely rare tumors comprising 1% of ovarian tumors. About 3% of all ovarian malignancies are primary ovarian sarcomas. Only 72 cases have been reported till date. A 57-year-old postmenopausal female presented with abdominal pain for the last 6 months. Ultrasonography and MRI revealed a heterogeneously enhancing solid lobulated mass in the left adnexa abutting the fundus of the uterus and bowel loops. The endometrial cavity was normal. Ovarian markers CA 125, CEA, CA 19.9, and all hematological parameters were within normal limits. LDH was near normal (284 IU/ml). The specimen was sent for frozen section and a diagnosis of malignant spindle cell lesion of ovary was rendered. Histopathology of the ovarian mass revealed intersecting fascicles of tumor cells consisting of ovoid to spindle-shaped cells having a moderate amount of cytoplasm. Bizarre and atypical cells were seen singly dispersed and in small aggregates along with the brisk mitotic activity. Focal areas of necrosis and hemorrhage were also noted. Immunohistochemistry showed strong positivity for smooth muscle actin and Caldesmon while focal positivity for Desmin and Epithelial Membrane Antigen (EMA) was noted. The lesion was negative for Inhibin, Calretinin, and CD 117 and S100. The final diagnosis of primary ovarian Leiomyosarcoma was given based on histopathology and Immunohistochemistry. PLMS of the ovary are rare incidental findings in postmenopausal women. These are highly malignant tumors and carry a poor prognosis. Hence, early diagnosis and surgical treatment with cytoreduction improve patient survival.

Age-related changes in macular vessels and their perfusion densities on optical coherence tomography angiography

Purpose: To evaluate age-related changes in macular vessels and their perfusion densities using optical coherence tomography angiography (OCTA). Methods: A total of 108 eyes of 54 healthy subjects between the age group of 11 to 60 years having unaided visual acuity of 20/20 were studied on spectral domain OCTS using 3 * 3 mm macula protocol. These subjects were divided into 5 groups; Group 1: 11–20 years, Group 2: 21–30 years, Group 3: 31–40 years, Group 4: 41–50 years, and Group 5: 51–60 years. An early treatment diabetic retinopathy study (ETDRS) grid overlay at the macula was used to calculate changes in different quadrants of the superficial retinal plexus. Results: A total of 98 eyes of 49 patients were considered for the final analysis. The vessel density values decreased from the second to the sixth decade in all four quadrants (except the third decade, where a slight increase was noted). The total vessel density decreased from 168 ± 78 mm-1 (group 1) to 131.47 ± 18.32 mm-1 (group 5). A similar reduction pattern in perfusion density was seen in each quadrant from the second to the sixth decade. The total perfusion density reduced from 309 ± 15.63% (group 1) to 283.05 ± 45.23% (group 5). The foveal avascular zone area was 0.18 ± 0.09 mm2 in group 1, 0.33 ± 0.13 mm2 in group 2, 0.30 ± 0.10 mm2 in group 3, 0.38 ± 0.05 mm2 in group 4, and 0.46 ± 0.06 mm2 in group 5. Conclusion: In our population, macular vessel density appears to decrease noticeably from the fourth decade onwards but a statistically significant decrease was observed only from the fifth decade onwards. However, it was not uniform along with all the quadrants. Similarly, the percentage of perfusion density dropped from the fourth decade but these values also varied among the different quadrants.

Novel ultrawide field fundus fluorescein angiographic findings in a patient of Takayasu arteritis on immunosuppression

A 35-year-old male, a case of Takayasu arteritis on treatment with oral prednisolone and methotrexate, revealed novel ultrawide field fluorescein angiographic features in bilateral eyes. The typical features of delayed arm-retina circulation time, lobulated areas of nonperfusion in choroid, cattle trucking of red blood cells in veins, and delayed arteriovenous transit time were nicely captured. Additionally, we noted peripheral perivascular hyperfluorescence and disc hyperfluorescence. We believe these findings could possibly be used as markers of disease activity. Lack of marked capillary nonperfusion areas and marked perivascular leakage probably signifies modulation of disease activity by the immunosuppressants.

Swept-source optical coherence tomography features of regressed macular retinoblastoma

Purpose: To describe the swept-source optical coherence tomography (SS-OCT) features of regressed macular retinoblastoma (RB). Methods: A cross-sectional observational study was carried out in 13 patients with regressed macular RB with good fixation in at least one eye. Fundus photography and SS-OCT were documented. High-resolution scans with good signal strength were selected. The types of clinical regression and SS-OCT characteristics of the regressed lesions (presence of vitreous detachment, intratumor schisis/cavitation, calcification, foveal dip, and OCT pattern) were noted. Results: Of the 13 eyes, 7 (53%) were group B, 4 (30%) were group C, and 2 (17%) were group D. Lesion involving fovea was seen in seven eyes (53%). On SS-OCT, the lesion was isodense to hyperdense in all cases. Three patterns of regressed RB were noted on OCT. Intralesion calcification was noted in eight cases. Subretinal fluid was not detected in any of the cases. Conclusion: SS-OCT is a useful technology to image and analyze cases of regressed macular RB including large lesions. SS-OCT system helps in successful imaging even in smaller children.

Coastline like peripheral chorioretinal degeneration as a suspected cause of nasal retinal dialysis

Retinal dialysis is mostly associated with blunt trauma or at times spontaneous. A patient presented to us with fresh rhegmatogenous retinal detachment with no telltale history or signs of trauma. The causative break was retinal dialysis noted on the superonasal periphery. A characteristic peripheral chorioretinal degeneration simulating a coastline almost extending six clock hours was seen in both the eyes. We have discussed this rare presentation and the possibilities of the association between this newly identified lesion and spontaneous retinal dialysis in the following case report.

Buckling surgery on a goat's eye: A simple technique to enhance residents' surgical skill

Buckling surgery is one of the common procedures performed by the retinal surgeons for visual rehabilitation at the earliest in cases of retinal detachment. The optimal surgical skill in this section can only be achieved with repeated practices and clinical experiences. Here, we describe an easy and inexpensive way to perform, practice, and refine surgical skills by demonstrating this complicated surgery in a simple manner on goat's eyes. The advantages of this technique are real-tissue handling experiences and repeatability of the procedure with almost similar practical implications. Thus, whenever feasible, every attempt should be made to refine the residents or budding ophthalmologists surgical skills by undertaking this technique in their routine curriculum.

Artificial intelligence in diabetic retinopathy: A natural step to the future

Use of artificial intelligence in medicine in an evolving technology which holds promise for mass screening and perhaps may even help in establishing an accurate diagnosis. The ability of complex computing is to perform pattern recognition by creating complex relationships based on input data and then comparing it with performance standards is a big step. Diabetic retinopathy is an ever-increasing problem. Early screening and timely treatment of the same can reduce the burden of sight threatening retinopathy. Any tool which can aid in quick screening of this disorder and minimize requirement of trained human resource for the same would probably be a boon for patients and ophthalmologists. In this review we discuss the current status of use of artificial intelligence in diabetic retinopathy and few other common retinal disorders.

Ultra-widefield fundus fluorescein angiography in pediatric retinal vascular diseases

Purpose: To describe the utility of RetCam ultra-wide-field fundus fluorescein angiography in pediatric retinal vascular diseases. Methods: A retrospective chart review was carried out in 43 eyes of 22 pediatric patients who were diagnosed or suspected to have a retinal vascular disease. Fluorescein angiography was carried out using the 130 degree lens of RetCam 3. Fluorescein angiography guided treatment (laser/cryotherapy) was carried out wherever required. Results: Diseases studied included - coats disease, familial exudative vitreoretinopathy, retinopathy of prematurity, congenital retinal folds, double optic nerve head, persistent fetal vasculature and incontinentia pigmenti. RetCam assisted fluorescein angiography was helpful in establishing a diagnosis in 4 patients (18%), in decision making regarding treatment in 18 patients (82%), in deciding need for retreatment in 5 patients (23%), helped in staging of disease in 5 patients (23%) and in detecting clinically subtle findings in 6 patients (27%). Conclusion: RetCam assisted FFA is extremely useful to document peripheral retinal vascular pathologies in pediatric patients and helps to take crucial therapeutic and retreatment decisions.

Assessment of macular vascular plexus density using optical coherence tomography angiography in cases of strabismic amblyopia

Purpose: To evaluate the superficial retinal vascular plexus density using optical coherence tomography angiography (OCTA) in cases of strabismic amblyopia. Methods: Ten eyes of 10 patients with purely strabismic amblyopia underwent detailed ocular evaluation followed by the assessment of the superficial retinal plexus vascular density using OCTA (Topcon DRI OCT Triton, Swept Source OCT, Topcon, Japan). Ten contralateral normal eyes of the same patients were considered as control. All these 20 eyes underwent a 4.5 × 4.5 mm cube scan OCTA centered at the fovea. Using the Topcon propriety software all 20 eyes were assessed for the capillary plexus density of the superficial retinal vascular plexus along the superior, inferior, nasal, and temporal quadrants centered at the fovea. The numerical values were statistically assessed using a paired t-test with respect to each quadrant between the normal and the pathological eyes. Results: The average age of patients was 16 years and eight patients were males. The mean superficial retinal vascular plexus density along the superior, inferior, nasal, and temporal quadrants in normal and pathological eyes were 49.25 ± 30.34 and 48.93 ± 2.85, 47.22 ± 4.11 and 47.37 ± 4.8, 45.54 ± 1.55 and 43.81 ± 4.21, and 46.26 ± 4.63 and 46.38 ± 5.40, respectively. Similarly, the capillary densities along the central were 17.84 ± 3.49 and 17.24 ± 2.44 in normal and pathological eyes. The differences among all these four quadrants and central area were not statistically significant (P-values > 0.05 for all four quadrants and central area) as compared with the normal eyes. Conclusion: The superficial retinal vascular plexus density of a 4.5 × 4.5 mm cube centered at the fovea of eyes of cases of strabismic amblyopia is similar to that of normal eyes.

Do oblate eyeballs exist? A rare case of rhegmatogenous retinal detachment in an elliptical globe

A patient, being a moderate myope with an axial length of 24.71 mm, presented to us with a fresh rhegmatogenous retinal detachment and marked peripheral chorioretinal degeneration. Difficulty in maneuvering with the standard 23 gauge vitrectomy cutter, inability to identify the break due to poor peripheral contrast, inadequate laser uptake, and an unusual large silicon oil fill (7.3 ml) were a few findings raising suspicion. Postoperative ocular ultrasonography showed an oblate eyeball with a relatively longer oblique axis (26.1 mm) as compared to the axial length confirming our suspicion. Oblateness should be suspected when the chorioretinal degenerations are more marked in the periphery as compared to the posterior pole. Intraoperative difficulties should be kept in mind while operating such cases.

An experimental study to evaluate safety/toxicity of intravitreal natalizumab

Purpose: The purpose of this prospective experimental study was to evaluate the safety/toxicity of ?4?1 integrin blockade in rabbit retina using its monoclonal antibody (Natalizumab). Methods: Twelve New Zealand albino rabbits were divided into three groups (n = 4). Unilateral intravitreal injections of three different concentrations of natalizumab were performed in every rabbit of each group (Group A: 0.625 mg, Group B: 1.25 mg, and Group C: 2.5 mg). Baseline electroretinogram (ERG) and fundus photography were performed prior to injection. At days 1, 7, and 21 postinjection, ERG and fundus photography of each eye were performed. At last follow-up, Group C animals with highest drug concentration were sacrificed and the enucleated eyes were evaluated for retinal toxicity using transmission electron microscopy (TEM). Results: No difference in ERG responses was observed in eyes injected with low and intermediate concentration of natalizumab between day 0 and day 21. Furthermore, rabbits injected intravitreally with highest dose showed reduction in amplitude of “a” wave (P = 0.0017) and a reduction in amplitude of “b” wave of ERG at day 21 (P = 0.0117). TEM revealed changes in the outer plexiform layer and inner nuclear layer, suggestive of toxicity primarily to the photoreceptor synaptic terminals and bipolar cells. Conclusion: Low-dose (0.625 mg) and intermediate-dose (1.25 mg) intravitreal injection of natalizumab appears safe for rabbit retina. However, functional and anatomical changes were observed in rabbit retina following a high-dose (2.5 mg) intravitreal injection of a monoclonal antibody blocking ?4?1 integrin.

Observer variation in quantitative assessment of retinal shortening with ultrasound in patients of total rhegmatogenous retinal detachment

Purpose: To evaluate the interobserver variation in the assessment of retinal length to choroidal length ratio (RCR) as a marker for proliferative vitreoretinopathy (PVR) in cases of rhegmatogenous retinal detachment (RRD). Methods: This was a double-masked, prospective study at a tertiary center. Ultrasound was used to calculate RCR in 50 eyes with total RRD by two observers. Both observers were trained after the first round of calculations, and all the calculations were repeated as before. Difference between the RCR values was stratified into four categories (<0.01, 0.01�05, 0.06�1, and >0.1) for descriptive analysis. A difference of 0.05 was set as the maximal limit for defining interobserver agreement. Correlation between RCR and interobserver difference was assessed. Results: The mean interobserver difference in RCR values was found to be 0.06 � 0.0 (P = 0.41) and was reduced to 0.04 � 0.02 (P = 0.81) following training. The interobserver difference was <0.1 in 82% of the cases before training and in 98% of cases after training. The worst interobserver agreement was noted in cases with RCR < 0.8, and there was a good negative correlation between RCR and interobserver difference (r = ?0.6, P ? 0.001). Conclusion: There is good interobserver agreement in assessing RCR with ultrasound in eyes with RRD, which improves further with training. RCR needs careful assessment in eyes with very low RCR. This technique may be useful in prognostication of surgical outcomes in cases with advanced PVR.