The Use of Hand-Held Digital Otoscope as a Basic Nasal Endoscopy Tool for Lacrimal Evaluation and Pedagogy Instrument.

PURPOSE: To investigate the utility of a hand-held digital otoscope for nasal endoscopy and as a pedagogy tool for residents and fellows in patients undergoing external dacryocystorhinostomy (DCR) surgery. METHODS: A digital otoscope (MS450-NTE, Teslong Inc., USA) comprising a digital screen device and a connectible camera probe was used for performing nasal endoscopy. Inspection of nasal cavities was performed pre-, intra-, and post-operatively in sequential patients with nasolacrimal duct obstruction, who underwent DCR or lacrimal probing. Images (1920 × 1080 pixels) and videos (1280 × 720 pixels) were captured. The device was also used for training residents and fellows in performing nasal endoscopy, and to teach basic concepts. RESULTS: The digital otoscope could be used for routine outpatient nasal examination and for performing minor procedures. 53.8% (n = 13) of ophthalmology trainees had never observed nasal endoscopy and 84.6% could not identify more than one major structure correctly prior to the current training. Post-training, all trainees could independently perform nasal endoscopy with the device and 76.9% identified all structures correctly. CONCLUSION: A digital otoscope with a camera probe is a handy tool for nasal endoscopy and pedagogy. Low-cost gadgets such as this device can effectively be used for performing outpatient nasal endoscopy when expensive endoscopes are unavailable and in peripheral healthcare centers.

Retinoblastoma: A review of the molecular basis of tumor development and its clinical correlation in shaping future targeted treatment strategies.

Retinoblastoma is a retinal cancer that affects children and is the most prevalent intraocular tumor worldwide. Despite tremendous breakthroughs in our understanding of the fundamental mechanisms that regulate progression of retinoblastoma, the development of targeted therapeutics for retinoblastoma has lagged. Our review highlights the current developments in the genetic, epigenetic, transcriptomic, and proteomic landscapes of retinoblastoma. We also discuss their clinical relevance and potential implications for future therapeutic development, with the aim to create a frontline multimodal therapy for retinoblastoma.

Giant facial congenital melanocytic nevus associated with conjunctival melanoma.

Giant congenital melanocytic nevi involving the face are benign lesions and malignant transformation to cutaneous melanoma involving the eyelid has been rarely reported. This report highlights the rare association of a giant facial melanocytic nevus and conjunctival primary acquired melanoses and melanoma.

Treatment outcomes of oral propranolol in the treatment of periocular infantile capillary hemangioma and factors predictive of recurrence and incomplete resolution: A multi-centric study.

AIM: This study aims to evaluate the treatment outcomes of periocular capillary hemangioma with oral propranolol (OP) and list the factors predictive of recurrence and incomplete resolution after treatment. METHODOLOGY: Data were collected by retrospective review of medical files of patients with infantile hemangioma (IH) treated with OP during January 2014-December 2019 from two tertiary eye institutes from India. Patients presenting with symptoms of IH with/without any history of prior treatment were included. All patients were started on OP with the dose of 2-2.5 mg/Kg body weight and continued till complete resolution or till the lesion reached a plateau response. Details about the ophthalmic examination at each visit and availability of imaging findings were noted down from the records. Primary outcome: Study the treatment outcome of patients treated with OP and discussed our observations regarding factors that may predict nonresponse, poor response, or recurrence. Secondary outcome: complications/side effects of therapy. Response to treatment was judged as fair, good, and excellent depending on resolution <50%, >50%, and complete resolution, respectively. Univariate analysis of factors related to treatment response was judged as fair, good, and excellent depending on resolution <50%, >50%, outcome and recurrence was studied using Mann-Whitney U test and Fisher's exact test. RESULTS: A total of 28 patients were included in the study, out of which 17 were female and 11 were male. The mean age of onset of the lesion was 1.08 (± 1.484) months, 11 being congenital in origin. The mean age at presentation was 4.15 (± 2.92) months. 46.43% (n = 13) of patients showed complete resolution, while 25% (n = 7) showed more than 50% reduction in lesion size. Fair response was noted in 28.57% (n = 8). The mean duration of follow-up after stopping OP was 17.7 (± 20.774) months. The recurrence rate noted was 14.28%. The factors which were associated with incomplete resolution were age at presentation >3 months, later age of appearance of the lesion, superficial lesions with no orbital involvement. Male gender and congenital lesions responded best to OP therapy. Minor complications were noted with a rate of 25% (n = 7). Younger age at presentation was more commonly associated with complications. CONCLUSION: OP is a safe and effective treatment for capillary hemangioma except for a smaller subset of patients who show suboptimal response to this drug. However, specific factors responsible for suboptimal response or recurrence after OP therapy remain elusive. Although not statistically significant, there was an increasing trend toward higher age at presentation, low birth weight, and superficial lesions with a poorer response. Furthermore, these factors along with the male gender were commonly associated with recurrence in our series. Larger prospective studies focused on evaluating clinical factors responsible for incomplete resolution and recurrence will help in prognosticating and suggesting alternative treatment regimes.

Intra-arterial chemotherapy in refractory and advanced intraocular retinoblastoma.

Purpose: To evaluate the efficacy of secondary and salvage intra-arterial chemotherapy (IAC) as a globe salvage treatment modality in advanced and refractory intraocular retinoblastoma. Methods: A retrospective chart review of advanced intraocular retinoblastoma (groups D and E International Classification of Retinoblastoma [ICRB] classification) patients refractory to intravenous chemotherapy (IVC) and undergoing IAC as the secondary and salvage treatment modality between December 2018 and June 2021 was carried out. All patients underwent the IAC procedure by super-selective ophthalmic artery catheterization and with triple-drug chemotherapeutic agents of melphalan, topotecan, and carboplatin. Data were collected about tumor regression, eye salvage, metastasis, and survival outcome at follow-up. Results: Out of 13 patients, 12 patients received secondary IAC after being primarily treated with IVC and focal therapies and one patient received rescue IAC after recurrence following primary IAC. Mean number of IAC cycles administered was 2. Overall, globe salvage rate was 53.84%, with a mean follow-up of 17.53 months (range 6-37 months), three patients had enucleation for residual tumor or tumor recurrence. One patient developed metastasis post enucleation and two patients who were lost to follow-up after enucleation advice for residual tumor developed orbital tumor extension and eventually died of metastasis. Conclusion: Secondary triple-drug IAC following failure of IVC, along with other adjunct treatment modalities might a be a cost-effective option for eye salvage in advanced intraocular retinoblastoma patients who refuse enucleation, with a globe salvage rate of 53.84%. It can also be an effective approach to improve treatment compliance and can help in addressing the barrier of treatment refusal when enucleation is advised.

Primary orbital yolk sac tumor presenting as fungating mass.

Primary yolk sac tumor of the orbit is a rare entity. Orbital involvement is usually seen in young children and proptosis is the commonest presentation. Aggressive orbital involvement and presentation as a fungating mass is rarely seen. We report a case of primary orbital yolk sac tumor with an aggressive presentation that responded well to systemic chemotherapy.

Single-Staged Surgical Correction of Eyelid Sequelae Along With Lid Margin Mucous Membrane Grafting in Stevens-Johnson Syndrome and Other Cicatricial Ocular Surface Diseases.

PURPOSE: The purpose of this study was to study the outcome of single-staged entropion surgery along with lid margin mucous membrane grafting for cicatrizing ocular surface disease. METHODS: Retrospective review of medical records of patients who underwent single-staged surgical correction of cicatricial entropion along with lid margin mucous membrane grafting for lid margin keratinization. RESULTS: Twenty-six eyes of 19 patients were studied. The mean age of patients was 42.5 years (standard deviation, SD-17.67), of which 7 patients were male and 12 were female. The most common disorder was Stevens-Johnson syndrome (SJS) sequelae (83.33%, n = 20), followed by mucous membrane pemphigoid (n = 4, 16.67%). The most common eyelid changes observed were cicatricial entropion in all 26 eyes (100%, n = 26), followed by trichiasis in 13 eyes (50%, n = 13). Lid margin keratinization was noted in all eyes. Postoperative improvement in corneal surface staining was noted in 70% of the patients (n = 13), no change in 20% of the patients (n = 4), and worsening of corneal surface staining in 10% of the patients (n = 2). Postoperative visual acuity improvement was noted in 50% of the eyes (n = 13), no improvement in 39% of the eyes (n = 10), and vision worsened in 12% of the eyes (n = 3). An entropion recurrence rate of 25% (n = 6) was observed over an average 10-month follow-up, whereas 75% (n = 20) reported no recurrence. CONCLUSIONS: Single-staged correction of eyelid cicatricial entropion with a lid margin mucous membrane graft (MMG) has promising outcomes in ocular surface diseases. It can decrease the need for multiple surgeries and provide symptomatic relief in patients with chronic cicatricial surface changes.

Does Multimodal Treatment Improve Eye and Life Salvage in Adenoid Cystic Carcinoma of the Lacrimal Gland?

PURPOSE: To evaluate the efficacy of multimodal treatment in adenoid cystic carcinoma (ACC) of the lacrimal gland. METHODS: A retrospective comparative case series of 40 consecutive patients with ACC of the lacrimal gland without systemic metastasis at the initial presentation and primarily managed by one of the 3 treatment protocols-surgery + external beam radiotherapy (EBRT) (group 1), surgery + EBRT + adjuvant chemotherapy (group 2), and neoadjuvant chemotherapy + surgery + EBRT + adjuvant chemotherapy (multimodal treatment) (group 3) at a tertiary care ocular oncology center. Local tumor control, eye salvage, and systemic metastasis were the primary outcome measures. RESULTS: The age ranged from 11 to 72 (mean ± SD, 36 ± 13; median, 36) years with 26 (65%) male and 14 (35%) female patients. Twelve (30%) patients belonged to group 1, 8 (20%) to group 2, and 20 (50%) to group 3. Primary surgery included tumor excision in 36 (90%) and orbital exenteration in 4 (10%). Chemotherapy composed of cisplatin + 5 fluorouracil (5FU) for 6 cycles in 28 (70%) patients. Extended-field stereotactic EBRT with a dose of 5,000-6,000 cGy included the entire pretreatment extent of the tumor with a 10-mm margin all around, superior orbital fissure, inferior orbital fissure, cavernous sinus, and temporal fossa. Mean duration of follow up after completion of treatment was 58 ± 26 (range, 29-180; median, 60) months. In all, local tumor recurrence occurred in 10 (25%) patients at a mean of 38 ± 23 (range, 12-120; median, 24) months. Local tumor recurrence was noted in 5 (42%) patients in group 1, 2 (25%) in group 2, and 3 (15%) in group 3. Overall, eye salvage was possible in 34 (85%) patients, with visual acuity >20/40 in 28 (82%). Systemic metastasis occurred in 10 (25%) patients at a mean of 53 ± 28 (range, 12-120; median, 43) months. Eight (67%) patients in group 1, 1 (13%) in group 2, and 1 (5%) in group 3 developed systemic metastasis. Six (15%) overall, 5 (42%) in group 1 and 1 (13%) in group 2, died with systemic metastasis. CONCLUSIONS: Multimodal treatment with sequential neoadjuvant chemotherapy, followed by surgery, extended-field stereotactic EBRT, and adjuvant chemotherapy seems relatively more effective in providing local tumor control and eye salvage and in minimizing the risk of systemic metastasis in ACC of the lacrimal gland.

Retinal Hemangioblastoma with Extraocular Extension: Report of Three Cases.

Retinal hemangioblastoma (RH) is the earliest and most common clinical manifestation in Von Hippel-Lindau (VHL) disease. RH can also present in isolation without any evidence of VHL. Clinical course of RH can be stationary or progress to exudation and chronic retinal detachment requiring surgical intervention. We report 3 cases of aggressive RH with extraocular extension in young males causing painful blind eye requiring enucleation. Two of our cases were bilateral involvement and had systemic manifestations of VHL. The third patient had unilateral involvement with no systemic evidence of VHL. This manifestation of RH is rarely reported. Two of our patients with VHL had early manifestations of RH and had undergone multiple cryotherapy sessions as well as retinal detachment surgery for exudative retinal detachment. This differential should be considered in vascular lesion arising from intraocular structures especially in diagnosed patients of VHL. The cases also highlight the aggressive behaviour and long-term progression of RH in some patients despite early treatment.

A study analyzing the health-related quality of life of retinoblastoma survivors in India.

Purpose: : Retinoblastoma (RB) is the commonest intraocular tumor in children. Despite high cure rates, data on health-related quality of life (HRQoL) of RB survivors are limited. This study aimed to analyze parent's perspective and self-report of HRQoL of RB survivors, using healthy siblings as controls. It also evaluated the impact of socio-economic status (SES), gender, disease laterality, treatment modality, duration since diagnosis, and visual outcomes, on HRQoL. Methods: Ninety-two RB survivors were enrolled in this observational, cross-sectional questionnaire-based study conducted at a tertiary care center. QoL was analyzed in four dimensions: physical, emotional, social, and school, using both self-report (for children >6 years) and parent proxy report (for children 2-18 years) using Pediatric Quality of Life Inventory™ (PedsQLTM) 4.0 Generic Core Scale. Seventy-seven healthy siblings served as controls. Results: The mean age of both cohorts was 5.7 years. Thirty-six (39%) patients had bilateral RB. Of the 92 survivors, 43 (47%) had undergone enucleation. The HRQoL of RB survivors was significantly lower compared to sibling controls (P < 0.01) in all four domains, the physical domain being most affected followed by social domain. Parents reported an inferior QoL than patient's self-report. Vision <6/18 in the best eye and enucleation had a negative impact on HRQoL whilst gender, disease laterality, duration since diagnosis and SES had no impact. Conclusion: : QoL assessment is often neglected but an important aspect of survivorship. Results of our study will help in formulating awareness of the domains affected and allow timely advocacy of initiatives for addressing each issue individually. Remedial measures aimed at optimizing QoL should be incorporated as part of their rehabilitation.

Malignant tumors of the eyelid in India: A multicenter, multizone study on clinicopathologic features and outcomes.

PURPOSE: To analyze data on eyelid malignancy in India, clinical and pathologic features, and outcomes. METHODS: A multicentre study, from oculoplastic practices in four geographic zones in India. The centers perform similar documentation and protocol-based management for eyelid tumors. Clinical features, pathology, American Joint Committee on Cancer (AJCC) class, management, and the outcomes were analyzed. RESULTS: The study included 129 patients, with slight female preponderance and mean age 62.7 years. The median delay to the consultation was 9 months. Rural patients traveled a mean distance of 115.2 km; there was no difference between the city and outstation patients in the delay to consultation or follow up. Pathology included 55/129 (42.6%) sebaceous gland carcinoma (SGC), 47/129 (36.4%) basal cell carcinoma (BCC), squamous cell carcinoma (SCC) in 15 (11.6%), and 12 (9.3%) other tumors. Commonest AJCC class was T2b/T3a in 80/111 (72%), invasion of the orbit was present in 16 (12.4%). Surgery with margin clearance was performed in 103. With a mean follow-up of 21.44 months, local recurrence and/or metastasis were seen in 12%. The diagnosis of SGC was strongly associated with adverse outcomes (odds ratio: 7.36). On multiple logistic regression analysis, diagnosis of SGC (P = 0.011) was significant in having adverse outcomes. CONCLUSION: The multicenter Indian data shows the highest prevalence of SGC, with the commonest AJCC class T2b. Most tumors were locally resectable at presentation. The histopathologic diagnosis of SGC is the factor strongly associated with adverse outcomes.

Clinical profile of thyroid eye disease and factors predictive of disease severity.

Purpose: To describe the clinical features of thyroid eye disease (TED) in patients presenting at a tertiary eye care centre in North India and to identify factors predictive of severe disease. Methods: This observational cross-sectional study involved clinical evaluation of all patients with TED who presented at the oculoplastic clinic based on the ITEDS VISA proforma. Risk factors for the severe disease were assessed using univariate and multivariate logistic regression. Results: A total of 106 patients (50 males, 56 females; mean age 41.30 ± 14.76 years) were identified during the study period, 46.23% hyperthyroid, 33.96% hypothyroid and 19.81% euthyroid. The proportion of the patients with hypothyroid was higher as compared with prior studies and most patients with hypothyroid had the mild disease (63.89%). Orbitopathy symptoms were the presenting feature leading to the diagnosis of systemic thyroid abnormality in 25% of the patients with hypothyroid and 59.18% of the patients with hyperthyroid, respectively (P < 0.05). Eyelid and orbitopathy signs were more common in the patients with hyperthyroid (51.2% and 87.7%) as compared with hypothyroid where the commonest presenting symptoms were related to dry eye (50.1%). Active disease was seen in 22.6% of the patients. Mild, moderate to severe and sight-threatening disease was seen in 54.7%, 37.7% and 7.5%, respectively. On multivariate analyses, hyperthyroid status and activity was associated with severe disease. Smoking was not associated with activity or severity. Conclusion: There is no significant difference in the gender profile of the patients with TED in this cohort. The patients with hypothyroid have a milder disease compared to the patients with hyperthyroid, and dry eye symptoms are the commonest presenting symptoms in hypothyroid subjects. Hyperthyroidism and activity were associated with severe and sight-threatening disease.

Ocular oncology practice guidelines during COVID-19 pandemic-An expert consensus.

The outbreak of rapidly spreading COVID-19 pandemic in December 2019 has witnessed a major transformation in the health care system worldwide. This has led to the re-organization of the specialty services for the effective utilization of available resources and ensuring the safety of patients and healthcare workers. Suspension of oncology services will have major implications on cancer care due to delayed diagnosis and treatment leading to irreversible adverse consequences. Therefore various oncology organizations have called for a continuation of cancer care during this crisis with diligence. The COVID-19 pandemic has forced the clinicians to transform the components of care from screening to outpatient care and primary management. The purpose of this article is to establish guidelines and recommendations for ocular oncology in the management of ocular tumors set by a multidisciplinary team of experts including ocular, medical and radiation oncologists, and pathologists. As the pandemic is evolving fast, it will require constant updates and reformation of health strategies and guidelines for safe and quality health care.