Showing papers by "Santosh G Honavar published in 2019"

Coats disease: An overview of classification, management and outcomes.

Abstract: Coats disease is an idiopathic retinal vascular disorder with retinal telangiectasia with intraretinal and/or subretinal exudation without appreciable retinal or vitreal traction. The condition is sporadic with no associated systemic abnormalities. Unilateral involvement in young males is the typical presentation with most cases being diagnosed in the first and second decade of life. Younger the patient, more severe is the presentation and poorer the visual outcome. The management varies with the stage of the disease. Over the years, we have shifted from enucleation to a more conservative approach for the treatment of Coats disease with laser photocoagulation, cryotherapy and surgery for retinal detachment achieving good outcomes. The anti-VEGF agents have come into the scene as important form of adjuvant treatment along with the traditional management options. This article describes the clinical features, underlying pathology, classification and staging, the complications and the management of Coats disease and gives an overview of the changing trends in treatment and outcomes spanning across five decades.

Study of Unilateral Retinoblastoma With and Without Histopathologic High-Risk Features and the Role of Adjuvant Chemotherapy: A Children’s Oncology Group Study

Abstract: PURPOSETo prospectively determine the prevalence of high-risk histopathologic features (HRFs) in patients with unilateral retinoblastoma who undergo enucleation and to evaluate the role of chemothe...

Circumscribed choroidal hemangioma: An overview of clinical manifestation, diagnosis and management.

Abstract: Circumscribed choroidal hemangioma is a benign vascular tumor which presents in middle-aged adults with progressive diminution of vision, metamorphopsia, floaters, and visual field defects. Diagnosis is based on the characteristic clinical features. It is an orange-red, usually solitary, tumor situated in the posterior pole. The visual symptoms are because of the associated subretinal fluid, cystoid macular edema, and, in long-standing cases, retinal pigment epithelium changes, subretinal fibrosis and retinoschisis. It must be distinguished from the more ominous amelanotic melanoma and choroidal metastasis. Diagnostic tools such as ultrasound, fundus fluorescein angiography, indocyanine green angiography, and optical coherence tomography are helpful in cases with diagnostic dilemma. Treatment is indicated in symptomatic cases. The management of choroidal hemangioma has evolved over the years beginning with laser photocoagulation to transpupillary thermotherapy, photodynamic therapy, plaque brachytherapy and external beam radiotherapy. No one therapeutic option holds superiority over the other. In this article, we review the epidemiology, clinical manifestations and treatment of the circumscribed variant of choroidal hemangioma.

Intraocular medulloepithelioma - A review of clinical features, DICER 1 mutation, and management.

Abstract: Intraocular medulloepithelioma is a nonhereditary neoplasm of childhood arising from primitive medullary epithelium. It most often involves the ciliary body. Most patients present between 2 and 10 years of age with loss of vision, pain, leucocoria, or conjunctival congestion. The mass appears as a grey-white ciliary body lesion with intratumoral cysts. Presence of a neoplastic cyclitic membrane with extension to retrolental region is characteristic. Secondary manifestations like cataract and neovascular glaucoma may be present in up to 50% and 60% patients, respectively. These could be the first signs for which, unfortunately, about 50% patients undergo surgery before recognition of the hidden tumor. Systemic correlation with pleuropulmonary blastoma (DICER1 gene) has been documented in 5% cases. Histopathology shows primitive neuroepithelial cells arranged as cords closely resembling the primitive retina. Histopathologically, the tumor is classified as teratoid (containing heteroplastic elements) and nonteratoid (containing medullary epithelial elements), each of which are further subclassified as benign or malignant. Retinoblastoma-like and sarcoma-like areas may be seen within the tissue. The treatment modality depends on tumor size and extent of invasion. For small localized tumors (≤3-4 clock hours), conservative treatments with cryotherapy, plaque radiotherapy, or partial lamellar sclerouvectomy (PLSU) have been used. Plaque brachytherapy is generally preferred for best tumor control. Advanced and extensive tumors require enucleation. Rare use of intra-arterial and intravitreal chemotherapy has been employed. Systemic prognosis is favorable, but those with extraocular extension and orbital involvement show risk for local recurrence and metastatic disease, which can lead to death.

The burden of uncorrected refractive error.

Abstract: Uncorrected refractive errors [defined as a presenting visual acuity of less than 6/12 in the better eye with an improvement of at least 0.2 logMAR (equivalent to two lines) after refraction][1] are the leading cause for moderate to severe vision impairment globally, and the second most common cause for blindness.[1,2] Among the global population with moderate or severe vision impairment (216 million) in 2015, the leading causes were uncorrected refractive error (116 million), cataract (52.6 million), age‐related macular degeneration (8.4 million), glaucoma (4.0 million), and diabetic retinopathy (2.6 million).[2] In the same year, the leading causes of global blindness (36.0 million) were cataract (12.6 million), uncorrected refractive error (7.4 million), and glaucoma (2.9 million).[2] Moderate to severe vision impairment cause by uncorrected refractive errors is expected to rise by about 10% to 128 million, and blindness attributable to uncorrected refractive errors is expected to increase by about 8% to 8.0 million by 2020.[2]

Do we need India-specific retinopathy of prematurity screening guidelines?

Abstract: Retinopathy of prematurity (ROP) is a major cause of preventable blindness in children.[1] It is estimated that of about 15 million children born preterm worldwide, about 53,000 develop sight-threatening ROP requiring treatment, and 20,000 suffer blindness or severe visual impairment.[2] More than 60% of preterm births occur in Africa and South Asia.[3] India accounts for the most preterm births in the world (3.5 million).[3] United Nations Children’s Fund estimates that 21 million newborns (15% of all births) have low birth weight (LBW).[4] India has the third highest incidence of LBW, with about 1.7 million weighing <2500 g and about 0.4 million <1500 g.[4] Crucially, premature birth and LBW predispose a newborn to develop ROP, for which India is evidently the hotbed.

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) of the eyelid: Clinicopathologic features, immunohistochemical findings and review of literature

Abstract: Sweat gland neoplasms are rare adnexal tumors that pose a diagnostic challenge for both, ophthalmologists and pathologists. Endocrine, mucin producing sweat gland carcinoma (EMPSGC), considered to be analogous to the solid papillary mammary carcinoma is one such tumor. It usually affects elderly, is more frequent in women and has a predilection for skin of the eyelid. Although it has an indolent clinical course, EMPSGC is believed to be a precursor of the invasive mucinous carcinoma and has a potential for local recurrence. We report a series of 10 biopsy-proven EMPSGCs with their immunohistochemical features and review the literature.

It's a doc's life – Workplace violence against doctors

Abstract: The recent incident at Nil Ratan Sircar Medical College and Hospital, Kolkata, is not the first of its kind, and going by the trend, it is unlikely to be the last. The medical fraternity today stands united against the increasing incidents of violence against doctors with vociferous nationwide protests. It is imperative that we look at the causes of this malady and try to find practical solutions for the same.

Driving blind - Should tests of visual function be mandatory for driving license?

Abstract: In 2017, there were 464,910 road traffic accidents in India and 147,913 fatalities, accounting for an accident every minute and a death every 3.5 minutes.[1] Causes of road traffic accidents are multifactorial. However, over 80% of accidents and fatalities are directly attributable to driver error.[2] Perceptual and cognitive functions that have an impact on driver safety and driver performance include visual functions, auditory skills, biomechanical skills, speed judgment and adaption, reaction time, and attention.[3,4] Visual functions contribute to about 90% of perceptual and cognitive inputs essential for safe driving, and hence are important assessable physical parameters to evaluate potential driver safety.[5]

Primary orbital low-grade fibromyxoid sarcoma – A case report

Abstract: An 18-year-old female presented with rapidly progressive proptosis of the left eye for one month and grade II relative afferent pupillary defect. Orbital imaging showed a well-defined homogenous extraconal mass in close relation to the lateral rectus muscle and extending up to the superior orbital fissure, associated with bony erosion. An incisional biopsy was performed, with the histopathology demonstrating stellate to spindle-shaped tumor cells (fibroblasts) embedded in a richly myxoid matrix. A diagnosis of low-grade fibromyxoid sarcoma (LGFS) was made. The patient was treated by stereotactic external beam radiotherapy. Here, we report a case of LGFS which, to the best of our knowledge, is the first at an orbital location.

Enucleation and Exenteration

Abstract: Enucleation and exenteration for the management of intraocular and orbital malignancies, respectively, was the standard treatment of care before the advent of radiotherapy and chemotherapy. Over the past few decades, enhanced knowledge on the biology of the tumors, advances in the histopathologic and cytological techniques, use of various diagnostic modalities for an early and a more precise diagnosis, implementation of protocol-based management, and use of multimodal treatment including chemotherapy, radiotherapy, immunotherapy, and conservative surgeries have greatly contributed to the reduction in the need for enucleation and exenteration. This paradigm shift toward less radical treatment has brought about a refinement in the absolute indications for enucleation and exenteration.

Gene expression analysis of retinoblastoma tissues with clinico-histopathologic correlation

Abstract: Purpose: Retinoblastoma (Rb) is the most common intraocular malignant tumor, which is not only unique but also has unraveled many novel aspects of tumor-suppressor genes. Genetic mutations of Rb, loss of phosphorylation, and many other factors resulted in uncontrolled cell division of the retinal cells resulting in tumor progression. In this study, we have analyzed the gene expression patterns of unilateral tumors (n = 11) in comparison to the normal-appearing retina (n = 2) from Rb patients who underwent enucleation for advanced Rb. With recent advances in the knowledge of the role of stem cells in these tumors, it is important to evaluate and understand the self-renewal signaling involved in these tumors. Here, in this study, we particularly aimed at evaluating the aberrant self-renewal signaling pathways in human Rb tumors and genes which show differential expression in cases with and without histologic risk factors (HRF). Materials and Methods: Freshly unfixed eyeballs (n = 11) were obtained. Normal-appearing retinas were pooled together (n = 2) and used as a control for microarray experiments. Total RNA was isolated from tumors and control tissues, and expression of genes was evaluated by hybridizing to expression arrays. Using real-time polymerase chain reaction (PCR), the results, thus, obtained were validated (for expression of N-Myc, HMGA2, LIN-28b, and Activin receptor 1C [ACVR1C]) in tissues compared to two control retinas latter obtained from enucleated Rb eyeballs without tumor. Furthermore, immunohistochemistry (IHC) was done on retrospective (n = 19) cases to confirm the expression of ACVR1C. Results: In Rb tumors, 5593 genes were upregulated and 4864 genes were downregulated (P ≤ 0.05 and fold change ≥1.5 folds). Changes in N-Myc, HMGA2, LIN28b, and ACVR1C expression detected by microarray were validated by real-time PCR. The analysis shows significant up-regulation of HMGA2 and its downstream regulator LIN-28b, which is involved in self-renewal pathway of fetal neural stem cells. ACVR1C is one of the markers, which shows differential expression between histological subtypes of tumors as evident in IHC. CBLB (P ≤ 0.05) and MAPK 8 (P ≤ 0.05) were shown to be highly upregulated in tumors without HRF compared to cases with HRF. Conclusions: This study showed up-regulation of genes involved in neural stem cell self-renewal and marginally in notch signaling. While other stem cell pathways such as Wnt and sonic hedgehog (SHH) pathways were upregulated in these tumors. Targeting these self-renewal pathways would aid in eliminating the resistant cells in this tumor and thus may help in treating the recurrence. Genes such as CBLB, MAPK 8, and ACVR1C can be used as potential biomarkers in this tumor to prognosticate cases with or without HRFs and differentiation of the tumors.

Back to the bedside.

Abstract: “He who studies medicine without books sails an uncharted sea, but he who studies medicine without patients does not go to sea at all.” – so said William Osler, and I must humbly agree. Sir Osler was one of the four founding professors of Johns Hopkins Hospital.[1] He is credited for creating the first residency program to bring medical students out of the lecture halls and to the bedside for an intensive clinical training.[1] Sir Osler unshakably believed that in a natural method of teaching, “the student begins with the patient, continues with the patient and ends his study with the patient, using books and lectures as tools, as means to an end.”

Preservation of retinoblastoma group E eyes with neovascular glaucoma using intravenous chemotherapy: risk factors and outcomes.

Abstract: Background/aim To report the outcomes of retinoblastoma group E eyes with neovascular glaucoma (NVG) treated conservatively with intravenous chemotherapy and investigate factors associated with eye salvage and secondary enucleation. Methods This is a retrospective, comparative, interventional case series. The outcome measures were life salvage, eye salvage and vision salvage. Results Of the 37 eyes managed by intravenous chemotherapy, secondary enucleation was necessary in 21 eyes (group 1) and eye salvage was possible in 16 eyes (group 2). A comparison of both groups revealed significant difference with group 1 demonstrating greater duration of symptoms (18.8 weeks vs 5.4 weeks, p=0.016), greater intraocular pressure (IOP) at presentation (36 mm Hg vs 30 mm Hg, p=0.044), greater increase in corneal diameter (1.52 mm vs 0.50 mm, p=0.013) and the presence of sterile orbital cellulitis (9 vs 1, p=0.023). Further, the risk factors for secondary enucleation by univariate analysis were duration of symptoms >10 weeks (p=0.003), presenting IOP >26 mm Hg (p=0.045), buphthalmos (p=0.014) and sterile orbital cellulitis (p=0.023) and by multivariate analysis were age at presentation >6 months (p=0.012) and buphthalmos (p=0.017). At a mean follow-up of 20.5 months, none of the patients in either group developed systemic metastasis. Conclusion For retinoblastoma group E eyes presenting with NVG, the chance of eye salvage with intravenous chemotherapy is better when the age at diagnosis is

Multifocal choroidal melanoma in oculodermal melanocytosis in an Asian Indian.

Abstract: A 51-year-old Asian Indian male with right oculodermal melanocytosis presented with a blurred visual acuity of 20/40. Upon fundus examination, he was discovered to have two independent pigmented choroidal melanomas in the temporal juxtapapillary region and inferiorly, in the region of trace clinical sectoral choroidal melanocytosis. The patient underwent enucleation, and on histopathology the two tumors were found to be in the area of choroidal melanocytosis, in continuity with each other. Multifocal choroidal melanoma is an exceedingly rare diagnosis. Ocular melanocytosis is a known predisposing factor for both unifocal and multifocal melanomas. Lifelong monitoring for uveal melanomas must be done in all patients with ocular melanocytosis.

Primary lacrimal gland teratoma in an adult: A clinicopathologic and radiological correlation.

Abstract: A 20-year-old male presented with a gradually growing mass in the left lateral brow region for 2 years. On imaging, the mass appeared to arise from the lacrimal gland, was cystic, without any bony abnormality. With a diagnosis of dermoid cyst, an excision biopsy was performed. The histopathology revealed an epithelium-lined cyst, with the wall of the cyst comprising dermal adnexa and inflammatory cells. The wall also contained fragments of skeletal muscle, nerve bundles, adipose tissue, exocrine and secretory acini, few blood vessels with occasional hair shafts, with the lacrimal gland in the periphery of the tumor. There were no immature elements, atypia, and malignant cells, thus confirming the diagnosis of a mature cystic teratoma. Primary lacrimal gland teratoma is a rare diagnosis and its presentation in adults is extremely uncommon.