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PURPOSE To discuss the association between ocular surface squamous neoplasia (OSSN) and Papillon-Lefevre syndrome (PLS) and present the long-term outcome in a patient with these diseases. METHODS Case report. RESULTS A 14-year-old boy presented with a raised pigmented mass lesion at the limbus in the right eye, which was clinically suggestive of OSSN. He also had palmoplantar hyperkeratosis and periodontosis suggestive of PLS. Excision biopsy of the lesion confirmed the diagnosis of OSSN. He was free of tumor recurrence for 2 years and was lost to follow-up thereafter. Seven years later, the patient presented with diffuse tumor recurrence with orbital extension in the right eye, regional lymph node metastasis, and ipsilateral parotid gland infiltration. The patient underwent exenteration of the right orbital contents, right parotidectomy, and radical neck dissection followed by concomitant external beam radiotherapy and systemic chemotherapy. Eleven years from initial presentation, the patient was detected to have OSSN in the contralateral eye and was managed by wide excision biopsy and adjuvant cryotherapy. One year after detection of OSSN in the left eye, the patient was found to have systemic metastases to the lungs and brain. The patient died of the disease within 3 months of detection of systemic metastasis. CONCLUSIONS OSSN in PLS is associated with poor prognosis.

Purpose To compare the surgical outcomes of ocular surface squamous neoplasia (OSSN) following wide excisional biopsy with and without primary simple limbal epithelial transplantation (p-SLET). Design Nonrandomized clinical study with historical controls. Methods setting: Single-institutional study. patients: Eight patients who underwent wide excisional biopsy of OSSN without p-SLET (historical controls) and 7 patients with p-SLET (cases). intervention: Wide excisional biopsy, p-SLET. main outcome measures: Limbal stem cell deficiency (LSCD). Results The tumor features of cases vs historical controls, including mean number of limbal clock hours affected by OSSN (6 vs 4; P = .12), mean tumor basal dimension (13 mm vs 8 mm; P = .11), and mean number of clock hours of corneoscleral limbal dissection owing to wide tumor excision (8 vs 7; P = .12), were comparable. The occurrence of partial LSCD in historical controls vs cases was 75% vs 0% ( P = .007) at a mean follow-up period of 12 months in both groups. Of these 6 historical controls that developed LSCD, pannus was noted in 1 (13%) and pseudopterygium extending onto the cornea in 5 (63%) patients. The mean number of clock hours of LSCD was 3 (median, 2; range, 2–6) in these historical controls. The mean time interval between surgical excision of OSSN and onset of LSCD was 8 weeks (median, 6 weeks; range, 6–12 weeks). Conclusion Corneoscleral limbal dissection of ≥6 clock hours during wide excision of OSSN can cause LSCD. Concomitant p-SLET after surgical excision of OSSN prevents LSCD in cases requiring extensive corneoscleral limbal dissection.

Choroidal hemangioma is a vascular tumor, which can either be circumscribed or diffuse. It most commonly occurs in the posterior pole as a reddish-orange choroidal mass. Ancillary testing with ultrasonography, indocyanine green angiography, and optical coherence tomography are useful in making an accurate diagnosis. The lesion can be simply observed when not associated with any signs of tumor activity. When associated with signs of tumor activity, the treatment options include photodynamic therapy, transpupillary thermotherapy, laser photocoagulation, plaque radiotherapy, external beam radiotherapy, proton beam radiotherapy, anti-vasoendothelial growth factors, or oral beta-blockers. The lesions respond well to treatment with excellent visual recovery.

PURPOSE: To study the high-risk histopathology features of retinoblastoma based on age at primary enucleation. METHODS: Retrospective study of 616 patients. RESULTS: The mean age at presentation and primary enucleation for retinoblastoma was 34 months (median, 28 months; range, 4 years of age at the time of enucleation. Bilateral retinoblastoma (34%; p 4 years. Based on 8th edition American Joint Committee on Cancer staging system, pT3 was less common in children ≤1 year of age (13%; p 4 years of age. Post-laminar optic nerve infiltration (6%; p = 0.02) and massive choroidal infiltration (9%; p = 0.04) was least common in children ≤1 year of age. Over a mean follow-up period of 52 months (median, 36 months; range

To study the demographic features, treatment, histopathology, and outcomes in patients of xeroderma pigmentosum (XP) with conjunctival melanoma. Retrospective case series. The median age at presentation was 18 years (range 9–30 years). There were three females and one male patient presenting with a median duration of symptoms of 3 months (range 1–60 months). The tumor was located in the bulbar conjunctiva in all 4 patients. All patients had corneal involvement by the tumor. The median tumor basal diameter was 7 mm (range 4–15 mm). Wide tumor excisional biopsy with alcohol keratoepithelectomy, cryotherapy to the free margins, and amniotic membrane grafting was done in three patients. One patient underwent orbital exenteration for extensive tumor. One patient also received adjuvant plaque brachytherapy for microscopic residual tumor. Over a median follow-up of 22 months (range 2–101 months), there were no recurrences, metastasis, or death. Conjunctival melanoma in XP is rare and manifests at a younger age.

To study the factors influencing the response to treatment with interferon alfa 2b (IFN) in ocular surface squamous neoplasia (OSSN)Methods: Retrospective study of 91 patientsResults: The mean age at presentation of patients with OSSN was 58 years (median, 60 years; range, 21 to 83 years). The mean number of clock hours of conjunctiva/cornea/limbus involvement by the tumor was 6 (median, 6; range, 1 to 12). The mean duration of topical IFN was 3 months (median, 3 months; range, 1 to 6 months) and the mean number of subconjunctival injections of IFN was 2 (median, 2; range, 0 to 6), till complete tumor regression or initiation of alternate treatment. Of 91 OSSN cases treated with IFN, 72 (79%) patients showed complete response to treatment, while 19 (21%) showed partial response displaying mean % tumor reduction of 34% (median, 20%; range, 5% to 90%). Patient demographics, immune status, disease chronicity, tumor location, or morphological pattern were not predictive of tumor response to IFN. The only factor predictive of incomplete response of OSSN to IFN was more than 6 clock hour involvement of ocular surface by OSSN (Clock hour involvement of ocular surface by OSSN determines the response to IFN. Interferon alfa 2b is an effective immunotherapy agent for tumors ≤6 clock hours of ocular surface in 82% cases and serves as an immunoreducing agent for larger tumors involving6 clock hours of ocular surface in 26% cases.

Purpose: To describe the clinical features, treatment, and outcomes of conjunctival melanoma in Asian Indians. Methods: Retrospective study of 42 patients. Results: The mean age at presentation of conjunctival melanoma was 43 years (median, 45 years; range, 9-78 years). There were 20 (48%) males and 22 (52%) females. Nineteen patients (45%) had a known history of a preexisting pigmented conjunctival lesion. Bulbar conjunctiva (n = 28; 67%) was the most common tumor epicenter, and medial ocular surface quadrant (n = 15; 36%) was more commonly involved. The mean tumor basal diameter was 12 mm (median, 10 mm; range, 4-30 mm), and the mean tumor thickness was 4 mm (median, 2 mm; range, 1-30 mm). Majority of the patients had a pigmented tumor (n = 33; 79%). The tumors arose de novo (n = 17, 41%) or were associated with conjunctival nevus (n = 9; 21%) or primary acquired melanosis (n = 16, 38%). Wide excisional biopsy, adjunctive cryotherapy, and amniotic membrane grafting were performed in 27 (71%) patients, 11 (29%) underwent orbital exenteration, and 4 were lost to follow-up prior to definitive treatment. Over a mean follow-up period of 24 months (median, 9 months; range

Retinoblastoma is a complex eye tumor manifesting in children below the age of 3 years. Over the years, the management of retinoblastoma has undergone drastic changes, thus improving the outcomes. The primary goal of management of retinoblastoma is life salvage, and secondary and tertiary goals include globe salvage and vision salvage, respectively, and thus improvement of the quality of life of the child. The choice of treatment of retinoblastoma depends on multiple factors including patients’ age, general condition, family desires, tumor laterality, tumor classification, tumor location, associated ocular features, radiological findings, and patient compliance to treatment. Smaller tumors can be managed with focal treatment like transpupillary thermotherapy or cryotherapy, while larger tumors need intravenous chemotherapy or intra-arterial chemotherapy. Enucleation is needed in very advanced tumors. External beam radiotherapy is rarely used except in cases with extraocular extension of retinoblastoma. Other supplemental treatments with subtenon’s chemotherapy, intravitreal chemotherapy, and plaque radiotherapy improve the outcomes further.

This case report demostrates an unusual occurence of peripunctal nevus and uveal melanoma, in which the clinical diganosis of uveal melanoma was masked by the atypical presentation as phthisis bulbi. Nevertheless, peculiar scleral pigment hinted at a possible intraocular tumour. The importance of meticulous clinical examination in assessment of ocular and periocular pigmented lesions is demonstrated. Further, clinicopathological differentials of correlation scleral pigmentation in diffuse necrotic uveal melanoma are illustrated.

A 49-year-old Asian Indian woman, with a previous history of biopsy proven stage IV primary lung adenocarcinoma with metastasis to liver, bones and central nervous system, presented with 1-month history of photopsia in right eye. She was on oral erlotinib since 6 months. Dilated fundus examination of right eye revealed a solitary dome-shaped brownish elevated lesion of approximately 1-disc diameter along the inferotemporal midperiphery with surrounding areas of hypopigmentation. Based on multimodal imaging, a diagnosis of resolved solitary unilateral choroidal metastasis from lung carcinoma in the right eye was made. In view of inactive and regressed choroidal metastasis, no intervention was mandated.

PURPOSE: To evaluate the efficacy of oral propranolol in the treatment of periocular infantile capillary hemangioma (CHI) based on the involvement of embryological facial placodes and their extent of anatomical involvement. METHODS: Retrospective study of 27 patients. RESULTS: The mean age at the presentation of periocular CHI was 4 months (median, 3 months; range

Mohammad Javed Ali,1 Swati Singh,1 Milind N Naik,1 Swathi Kaliki,2 Tarjani Vivek Dave1 1Govindram Seksaria Institute of Dacryology, 2The Operation Eyesight Universal Institute for Eye Cancer, LV Prasad Eye Institute, Hyderabad, Telangana, India Aim: The aim of this study was to report the preliminary experience with the techniques and utility of navigation-guided, 3D, computed tomography–dacryocystography (CT-DCG) in the management of secondary acquired lacrimal drainage obstructions.Methods: Stereotactic surgeries using CT-DCG as the intraoperative image-guiding tool were performed in 3 patients. One patient had nasolacrimal duct obstruction (NLDO) following a complete maxillectomy for a sinus malignancy, and the other 2 had NLDO following extensive maxillofacial trauma. All patients underwent a 3D CT-DCG. Image-guided dacryolocalization (IGDL) was performed using the intraoperative image-guided StealthStation™ system in the electromagnetic mode. All patients underwent navigation-guided powered endoscopic dacryocystorhinostomy (DCR). The utility of intraoperative dacryocystographic guidance and the ability to localize the lacrimal drainage system in the altered endoscopic anatomical milieu were noted.Results: Intraoperative geometric localization of the lacrimal sac and the nasolacrimal duct could be easily achieved. Constant orientation of the lacrimal drainage system was possible while navigating in the vicinity of altered endoscopic perilacrimal anatomy. Useful clues with regard to modifications while performing a powered endoscopic DCR could be obtained. Surgeries could be performed with utmost safety and precision, thereby avoiding complications. Detailed preoperative 3D CT-DCG reconstructions with constant intraoperative dacryolocalization were found to be essential for successful outcomes.Conclusion: The 3D CT-DCG-guided navigation procedure is very useful while performing endoscopic DCRs in cases of secondary acquired and complex NLDOs. Keywords: CT-dacryocystography, image guidance, navigation, lacrimal drainage, nasolacrimal duct obstruction, powered endoscopic DCR

PURPOSE To develop a standardized and universally reproducible grading scale for artistic outcome of a custom ocular prosthesis. METHODS A retrospective review of photographs of patients with custom ocular prosthesis. From the ocularistry database of a tertiary eye care hospital, photographs of patients with custom ocular prosthesis (30 random photos with additional 18 repetitions, total n = 50) were selected. Two independent oculoplastic surgeons, on the basis of characteristics of limbus, vascularity, and pigmentation, graded these photos. The photos were adjusted for confounders like magnification and illumination, and both graders used the same display system. The surgeons were blinded to each other. The characteristics were graded on a scale of 0 to 2 and total score was calculated for each photo. Inter-observer and intra-observer agreement was calculated using the kappa statistic. RESULTS The inter-observer kappa coefficient for the three characteristics (95% confidence interval) were as follows: limbus 0.7 (0.55-0.85), vascularity 0.76 (0.58-0.94), and pigmentation 0.6 (0.34-0.85). The intra-observer kappa coefficient for the first surgeon was as follows: limbus 0.88 (0.7-0.95), vascularity 0.88 (0.73-0.95), and pigmentation 0.82 (0.54-0.93). The intra-observer kappa coefficient for the second surgeon was as follows: limbus 0.8 (0.44-0.93), vascularity 0.55 (0.13-0.8), and pigmentation 0.76 (0.6-0.84). CONCLUSIONS A good intra- and inter-observer agreement was demonstrated. In view of no existing grading for prosthesis, this is a unique system to objectively grade the outcome of custom ocular prosthesis and can be incorporated in ocularistry teaching modules for standardization of outcomes.

Purpose: To identify the clinical features predictive of choroidal infiltration by retinoblastoma on histopathology and to report the outcome in these patients. Methods: Retrospective study. Results: Of the 403 patients who underwent primary enucleation for retinoblastoma, 113 patients had choroidal tumor infiltration and 290 patients had no choroidal tumor infiltration. There was a higher incidence of metastasis and related death in the choroidal tumor infiltration group compared to the no choroidal tumor infiltration group (4% vs 1%; P = .02). On multivariate analysis, the clinical features predictive of histopathologic massive choroidal infiltration included prolonged duration of symptoms for more than 6 months (hazard ratio [HR] = 3.04; P = .001) and secondary glaucoma (HR = 2.24; P = .005). Conclusions: In this study, the patients with retinoblastoma with prolonged duration of symptoms (> 6 months) had a three-fold greater risk and those with secondary glaucoma at presentation had a two-fold greater risk of massive choroidal tumor infiltration. [ J Pediatr Ophthalmol Strabismus . 2016;53(6):349–356.]

Mohammad Javed Ali,1 Swati Singh,1 Milind N Naik,1 Swathi Kaliki,2 Tarjani Vivek Dave1 1The Institute of Dacryology, 2The Operation Eyesight Universal Institute for Eye Cancer, L.V. Prasad Eye Institute, Hyderabad, India Purpose: The aims of this study were to report the preliminary experience of using telescopes, which were enabled for navigation guidance, and their utility in complex endoscopic lacrimal surgeries. Methods: Navigation enabling of the telescope was achieved by using the AxiEM™ malleable neuronavigation shunt stylet. Image-guided dacryolocalization was performed in five patients using the intraoperative image-guided StealthStation™ system in the electromagnetic mode. The “look ahead” protocol software was used to assist the surgeon in assessing the intraoperative geometric location of the endoscope and what lies ahead in real time. All patients underwent navigation-guided powered endoscopic dacryocystorhinostomy. The utility of uninterrupted navigation guidance throughout the surgery with the endoscope as the navigating tool was noted. Results: Intraoperative geometric localization of the lacrimal sac and the nasolacrimal duct could be easily deciphered. Constant orientation of the lacrimal drainage system and the peri-lacrimal anatomy was possible without the need for repeated point localizations throughout the surgery. The “look ahead” features could accurately alert the surgeon of anatomical structures that exists at 5, 10 and 15 mm in front of the endoscope. Good securing of the shunt stylet with the telescope was found to be essential for constant and accurate navigation. Conclusion: Navigation-enabled endoscopes provide the surgeon with the advantage of sustained stereotactic anatomical awareness at all times during the surgery. Keywords: telescope, endoscope, image guidance, navigation, lacrimal surgery, powered endoscopic DCR

Purpose To describe clinical presentation and histopathological features of a nodulo-ulcerative variant of ocular surface squamous neoplasia (OSSN). Methods Retrospective study. Results Six patients were identified with a nodulo-ulcerative variant of OSSN. Mean age at presentation was 56 years. One patient was immunosuppressed because of human immunodeficiency virus infection. The initial misdiagnosis was necrotizing scleritis/sclerokeratitis (n = 4), intraepithelial neoplasia (n = 1), and chalazion (n = 1). The mean delay in diagnosis as OSSN was 6 months. The lesion involved bulbar conjunctiva in 5 patients and tarsal conjunctiva in 1 patient. The mean maximum tumor dimension was 16 mm, and the mean area of conjunctivoscleral/conjunctivotarsal necrosis was 8 mm. The primary treatment for nodulo-ulcerative OSSN included excisional biopsy (n = 2), plaque radiotherapy (n = 1), and orbital exenteration (n = 3). Tumor recurrence was noted in two patients necessitating orbital exenteration. At last follow-up, globe salvage could be achieved in only 1 case. Histopathology showed invasive conjunctival squamous cell carcinoma in all cases with lesions being well-differentiated (n = 4), moderately differentiated (n = 1), and with mucoepidermoid differentiation (n = 1). Underlying scleral/tarsal tumor infiltration was evident in all 6 (100%) cases. Intraocular tumor extension was evident in 3 cases infiltrating the iris (n = 2), ciliary body (n = 3), and choroid (n = 3). Orbital tumor extension was evident in 2 cases because of tumor recurrence. No metastasis or death was recorded at a mean follow-up of 26 months. Conclusions The nodulo-ulcerative variant of OSSN is rare and is most often misdiagnosed as necrotizing scleritis/sclerokeratitis causing a delay in the diagnosis. It is an aggressive lesion with increased occurrence of intraocular or orbital tumor extension.

The aim of this study was to illustrate the techniques and usefulness of navigation-guided computed tomography angiography in ophthalmic plastic surgery.Stereotactic surgeries using computed tomography angiography as the intraoperative image-guiding tool were performed in 3 patients. One patient had widespread eyelid and orbital vascular malformation and the other 2 had orbital mass lesions located in crucial vascular areas. Two procedures used the external approach and one was performed using the endoscopic orbital approach. All the surgeries were performed using the intraoperative image-guided StealthStation system in the electromagnetic mode. The usefulness of intraoperative angiographic anatomical guidance and the ability to anticipate and avoid vascular injuries at crucial phases of surgery were noted.Intraoperative geometric localization of feeder vessels to a lesion could be easily achieved. The cauterization of the direct feeder to the lesion reduced the much anticipated blood loss while operating a widespread vascular malformation. Orbital mass lesions with vessels in the vicinity could be operated upon with utmost vascular safety and precision, thereby avoiding complications. Detailed preoperative navigation-guided planning of risk structures with intraoperative distance control was found to be essential for successful outcomes.Computed tomography angiography-guided navigation surgery is very useful in cases with complex vascular malformations and while operating in the vicinity of crucial vascular structures in the orbit.

To identify the clinical features predictive of any optic nerve infiltration and postlaminar optic nerve infiltration by retinoblastoma on histopathology and to report the outcome (metastasis and death) in these patients.Retrospective study.Of the 403 patients who underwent primary enucleation for retinoblastoma, 196 patients had optic nerve tumor infiltration (Group 1) and 207 patients had no evidence of optic nerve tumor infiltration (Group 2). Group 1 included patients with prelaminar (n = 47; 24%), laminar (n = 74; 38%), and postlaminar tumor infiltration with or without involving optic nerve transection (n = 74; 38%). Comparing Group 1 and Group 2, the patients in Group 1 had prolonged duration of symptoms (6 months) (16% vs. 8%; P = 0.02) and were associated with no vision at presentation (23% vs. 10%; P = 0.01), higher rates of secondary glaucoma (42% vs. 12%; P0.0001), iris neovascularization (39% vs. 23%; P0.001), and larger tumors (mean tumor thickness, 12.8 mm vs. 12 mm; P = 0.0001). There was a higher prevalence of metastasis in Group 1 than in Group 2 (4% vs. 0%; P = 0.006). On multivariate analysis, clinical features predictive of any optic nerve tumor infiltration secondary glaucoma (hazard ratio = 5.38; P0.001) and those predictive of postlaminar optic nerve tumor infiltration included iris neovascularization (hazard ratio = 2.66; P = 0.001) and secondary glaucoma (hazard ratio = 3.13; P0.001).In this study, clinical features predictive of any optic nerve tumor infiltration included secondary glaucoma and those predictive of postlaminar optic nerve tumor infiltration included iris neovascularization and secondary glaucoma. Despite adjuvant treatment in those with postlaminar optic nerve tumor infiltration, metastasis occurred in 8% of patients.

Purpose: The purpose was to study the efficacy of interferon alpha 2b (INF α2b) in the treatment of ocular surface squamous neoplasia (OSSN) and analyze its cost-effectiveness in India. Study Design: This was a retrospective study of thirty patients with OSSN treated with topical INF α2b (1 MIU/cc) ± perilesional INF α2b (5 MIU/cc). Results: The tumor involved cornea (n = 9, 30%), conjunctivo-limbal-corneal surface (n = 19, 63%), or bulbar conjunctiva (n = 2, 7%). The mean basal dimension of the tumor was 16 mm. The tumors belonged to Tis (n = 6, 20%) or T3 (n = 24, 80%) based on the American Joint Committee Classification, 7th edition. In the six patients with Tis, three cycles of topical INF α2b were used for immunoprevention. In the remaining 24 patients, INF α2b was advised for immunoreduction, but served as immunotherapy with 100% tumor regression in 22 (92%) cases, and resulted in 95% immunoreduction in 2 (6%) cases. Complete tumor regression by immunotherapy (n = 22) was achieved with a mean number of three topical INF α2b cycles and two perilesional injections. All these 22 patients received three additional topical INF α2b cycles after complete tumor regression. For immunoreduction (n = 2), both patients received six cycles of topical INF α2b which was three perilesional INF α2b injections. The mean total treatment cost per patient with INF α2b was INR 9164 ($US 137). Based on maximum basal diameter of tumor at presentation, the mean total treatment cost per patient with INF α2b was INR 4866 ($US 73) for eyes with microscopic evidence of tumor residue (n = 6), INR 9607 ($US 143) for tumors ≤10 mm (n = 13), and INR 10,985 ($US 164) for tumors >10 mm (n = 11), with two patients needing additional surgical excision for complete tumor control. Conclusion: INF α2b can be used for immunoreduction, immunotherapy, or immunoprevention of OSSN. INF α2b is a cost-effective treatment modality for OSSN at an average total treatment cost of INR 9164 ($US 137) per patient.

An 11-year-old boy presented to the outpatient department of a tertiary eye hospital with progressively growing raised pigmented lesions temporally in both eyes (BE) for 2 years. He consulted elsewhere 6 months prior where excision of Left Eye (LE) lesion was performed suspecting pterygium.

Clinical diagnosis is always challenging in cases with atypical presentation. Herein, we present two cases which masqueraded as ocular infection/inflammation on presentation, were clinically suspicious for retinoblastoma, and histopathology revealed the diagnosis of retinal dysplasia. Case 1 had left corneal perforation with anterior chamber exudates on presentation. On ultrasound B-scan, ill-defined mass was noted, raising a suspicion of malignancy. MRI showed dilated ventricles with midline shift. Vitreous cytology was inconclusive. Enucleation was performed as malignancy could not be ruled out. Histopathology revealed detached retina with dysplastic rosettes in addition to inflammation and multinucleate giant cell reaction. Case 2 presented with right eye anterior chamber pseudohypopyon. Fundus examination revealed diffuse vitreous haze and a suspicious mass in the retinal periphery raising suspicion for retinoblastoma. Histopathology revealed the diagnosis of retinal dysplasia.

A 32-year-old Asian-Indian woman presented with painless diminution of vision in her left eye (oculus sinister (OS)) and fundus examination OS revealed a large dome-shaped orangish-red subretinal lesion at the macula along with a smaller lesion on the superotemporal arcade. Indocyanine green angiography and optical coherence tomography features confirmed the diagnosis of multifocal circumscribed choroidal hemangioma (CCH). There was no evidence of Sturge-Weber syndrome. CCH is usually solitary with only one reported case of multifocal CCH in the literature.

Sebaceous gland carcinoma (SGC) of the eyelid is one of the leading peri-ocular eyelid malignancies in Asian-Indians. It usually affects elderly women and has a high rate of local recurrence, regional and distant metastasis. This is partly attributable to a delay in clinical diagnosis since it mimics more benign conditions of the eyelid like chalazia. To the best of our knowledge, this is the first case report of a peri-punctal SGC. Atypical location and the young age of the patient resulted in a relatively conservative excisional biopsy under frozen section control and eyelid reconstruction with a favorable outcome.

PURPOSE: To describe the epidemiology of ocular and periocular tumors in patients presenting to a multi-tier ophthalmology hospital network in India using the electronic medical records (EMRs) system. METHODS: A retrospective, observational, referral, hospital-based study of 1,142,098 patients. The data were collected using the in-house developed EyeSmart EMR system. RESULTS: During a 6-year study period, 9633 (0.8%) new patients were diagnosed with eye tumors. Of the 9633 patients, 5209 (54%) were male and 4424 (46%) were female. Of all tumors, 6372 (65%) were benign, 282 (3%) were pre-malignant, and 3089 (32%) were malignant in nature, respectively. Overall, the three most common tumors included retinoblastoma (n = 1167, 12%), ocular surface squamous neoplasia (n = 957, 10%), and conjunctival nevus (n = 903, 9%). The three most common benign tumors included conjunctival nevus (n = 903, 9%), eyelid nevus (n = 358, 4%), and orbital dermoid cyst (n = 344, 4%). The three most common malignant tumors included retinoblastoma (n = 1167, 12%), ocular surface squamous neoplasia (n = 957, 10%), and sebaceous gland carcinoma (n = 202, 2%). The most common tumor in 0–10 years' age group was retinoblastoma (n = 1163, 42%), 11–30 years was conjunctival nevus (n = 408, 16%), and > 30 years was ocular surface squamous neoplasia (n = 801, 17%), respectively. CONCLUSIONS: The present study results indicate the incidence and distribution of ocular and periocular tumors in a large cohort in India. Retinoblastoma is the most common tumor encountered in a referral-based comprehensive ophthalmic oncology practice in India. The use of EMRs enables to capture the structured information and big data analysis of the same.

The 2018 Ocular Oncogenesis and Oncology Conference was held through a partnership of the Association for Research in Vision and Ophthalmology (ARVO) and the Champalimaud Foundation. Twenty-one experts from international ocular oncology centers, from the Champalimaud Clinical Centre and the Champalimaud Foundation Cancer Research Program, and from patient advocacy organizations, delivered lectures on subjects that ranged from global ocular oncology, to basic research in mechanisms of ocular malignancy, to clinical research in ocular cancers, and to anticipated future developments in the area. The scientific program of the conference covered a broad range of ocular tumors-including uveal melanoma, retinoblastoma, ocular surface tumors, and adnexal and intraocular lymphomas-and pathogenesis and management were deliberated in the context of the broader systemic cancer discipline. In considering the latest basic and clinical research developments in ocular oncogenesis and oncology, and providing the opportunity for cross-talk between ocular cancer biologists, systemic cancer biologists, ocular oncologists, systemic oncologists, patients, and patient advocates, the forum generated new knowledge and novel insights for the field. This report summarizes the content of the invited talks at the 2018 ARVO-Champalimaud Foundation Ocular Oncogenesis and Oncology Conference.

To review the indications of enucleation in Asian Indian patients and study the trend over the 22-year period.Retrospective study of 2009 patients who underwent enucleation.The mean age at presentation of patients who underwent enucleation was 155 months The histopathology diagnosis included a benign tumor (n = 22, 1%), malignant tumor (n = 1472, 73%), acute trauma (n = 93, 5%), retinal vascular disease (n = 50, 3%), inflammatory/infective pathology (n = 33, 2%), or other miscellaneous/non-specific diagnosis (n = 460, 23%). There was a good correlation between the clinical and histopathology diagnoses at 96%. The most common indication for enucleation in young patients ( ≤ 20 years) was retinoblastoma (n = 1257, 82%; p 0.001), atrophic bulbi or phthisis bulbi (n = 163, 39%; p 0.001) in middle-age adults, and uveal melanoma (n = 25, 42%; p 0.001) in older adults. Over the years, there was a decreasing trend of enucleations for atrophic bulbi/phthisis bulbi/painful blind eye (33% from the years 1996 through 2000 to 7% from 2010 to 2018; p 0.001) and acute trauma (3% from the years 1996 through 2000 to 1% from 2010 to 2018; p 0.001) and an increasing trend for intraocular tumors including retinoblastoma (56% from the years 1996 through 2000 to 73% from 2010 to 2018; p = 0.01) and uveal melanoma (3% from the years 1996 through 2000 to 11% from 2010 to 2018; p 0.006).In Asian Indian population, malignant tumors remain the most common indication for enucleation in young and older patients, while desire for better cosmesis with customized ocular prosthesis is the main indication for enucleation in middle-age adults.摘要: 目的: 通过回顾亚洲印度地区的眼球摘除术适应症, 研究眼球摘除术适应症未来22年的发展趋势。方法: 回顾性分析2009例行眼球摘除术的患者。结果: 眼球摘除术患者平均年龄155个月, 病理组织学诊断包括良性肿瘤(n=22, 1%)、恶性肿瘤(n=1472, 73%)、急性创伤(n=93, 5%)、视网膜血管疾病(n=50, 3%)、炎症/感染病理(n=33, 2%)或其他混杂/非特异性诊断(n=460,23%)。96%的患者临床诊断与病理组织学诊断有较好的相关性。年轻患者(≤20岁)最常见的眼球摘除术适应症是视网膜母细胞瘤(n=1257, 82%;p 0.001);中年人常见的眼球摘除术适应症是眼球萎缩或眼结核(n=163,39%;p 0.001);老年人常见的眼球摘除术适应症是葡萄膜黑色素瘤(n=25,42%;p 0.001)。近年来, 眼球萎缩/眼结核/疼痛性失明行眼球摘除术者 (1996年-2000年 33%, 2010年-2018年7%)和急性创伤行眼球摘除术者(1996年-2000年3%, 2010年-2018年1%;p 0.001)均呈现下降趋势。而眼内肿瘤行眼球摘除术者呈上升趋势, 其包括视网膜母细胞瘤(1996年-2000年 56%, 2010年-2018年 73%;p=0.01)和葡萄膜黑色素瘤(1996年-2000年3%, 2010年-2018年11%;p 0.006)。结论:在亚洲印度地区人群中, 恶性肿瘤仍然是青年和老年患者行眼球摘除术最常见的适应症, 而对个性化眼球假体更好的晶状体形态的渴望是中老年人行眼球摘除术的主要指征。.

Supplemental Material available at https://www.ajo.com/.; International audience; PURPOSE: To compare clinical, optical coherence tomography (OCT), and fundus autofluorescence (FAF) characteristics of peripapillary vs macular variants of combined hamartoma of the retina and retinal pigment epithelium (combined hamartoma). DESIGN: Retrospective observational, comparative case series. METHODS: SETTING: Multicenter collaborative study. STUDY POPULATION: Fifty eyes with a clinical diagnosis of combined hamartoma. OBSERVATIONAL ANALYSIS: A comparative analysis of color fundus photographs (CFPs), OCT, and FAF was performed for peripapillary and macular variants of combined hamartoma. MAIN OUTCOME MEASURES: Pigmentation and OCT features of macular and peripapillary combined hamartoma. RESULTS: The review of imaging from 50 eyes of 49 patients diagnosed with combined hamartoma identified 18 (36%) peripapillary lesions, 27 (54%) macular lesions, and 5 (10%) peripheral lesions. A comparative analysis of peripapillary vs macular combined hamartoma identified differences in the following features: lesion pigmentation on CFPs corresponding to hypoautofluorescent FAF (88% vs 0%, P < .001) and OCT features of full-thickness involvement (88% vs 3%, P < .001), preretinal fibrosis (27% vs 81%, P < .001), maxi peaks (5% vs 88%, P < .001), intraretinal cystoid spaces (72% vs 40%, P < .038), outer plexiform layer involvement (5% vs 96%, P < .001), ellipsoid zone disruption (83% vs 3%, P < .001), RPE disruption (77% vs 3%, P < .001), and choroidal neovascularization (16% vs 0%, P = .028). CONCLUSIONS: This comparative analysis identified a higher frequency of pigmentation with hypoautofluores-cence, full-thickness retinal involvement, intraretinal cystoid spaces, ellipsoid zone disruption, RPE disruption, and choroidal neovascularization in peripapillary combined hamartoma. These findings suggest that lesions occurring near or at the optic nerve are associated with a more severe degree of pigmentary changes and retinal disruption than those located in the macula.

Purpose We studied the incidence, treatment, and outcome of ocular and periocular tumors in patients with xeroderma pigmentosum (XP). Design Retrospective case series. Methods This single-institution study included 120 patients with XP who underwent intervention with excisional biopsy, enucleation, or orbital exenteration. The primary outcome measures were the occurrence of eyelid or ocular surface tumor, globe salvage, locoregional and systemic metastasis, and death. Results The mean age at presentation was 19 years. A family history of XP was present in 32 (27%) patients. Over a mean follow-up of 61 months, 34 (28%) patients developed no ocular/adnexal tumor, 86 (72%) developed ocular surface malignancy, 15 (13%) developed eyelid malignancy, and 22 (18%) developed other head and neck malignancies. Of the 86 patients with ocular surface malignancy, 48 (56%) had unilateral tumor and 38 (44%) had bilateral tumors. Invasive squamous cell carcinoma (n = 51, 41%) was the most common ocular surface tumor. Of the 15 patients with eyelid tumors, 14 (93%) had unilateral tumor and 1 (7%) had bilateral involvement. Basal cell carcinoma (n = 8, 50%) was the most common eyelid tumor. There were events of ocular surface tumor recurrence (n = 55 eyes, 44%), eyelid tumor recurrence (n = 5 eyes, 31%), locoregional lymph node metastasis (n = 3, 2%), systemic metastasis (n = 1, 1%), and death (n = 1, 1%). Overall, globe salvage was achieved in 119 (99%) patients (both eyes were salvaged in 92 [76%] patients and at least 1 eye was salvaged in 27 [23%] patients). Conclusion XP is frequently associated with ocular surface, eyelid, and other head and neck malignancies. Lifelong follow-up is mandatory in these patients.

Purpose To study the clinical presentation and treatment outcome of patients who underwent inadvertent intraocular surgery before the diagnosis of retinoblastoma. Design Retrospective study of 14 patients who had undergone an inadvertent intraocular surgery before the diagnosis of retinoblastoma. Results The mean age at presentation to the ocular oncology clinic was 69 months. The most common initial misdiagnosis was endophthalmitis (n = 4). The most common inadvertent intraocular surgeries were pars plana vitrectomy (n = 6) with/without lensectomy and evisceration with/without previous pars plana vitrectomy (n = 5). The mean interval between intraocular procedure and initiation of treatment for retinoblastoma was 7 months. At presentation in the oncology clinic, the tumor was intraocular (n = 3), with extrascleral tumor extension (n = 11), and/or optic nerve tumor extension (n = 5). All patients were started on multimodal treatment including 12 cycles of high-dose systemic chemotherapy, enucleation or orbital exenteration, and orbital external beam radiotherapy. Over a mean follow-up period of 27 months, 8 (57%) patients died because of progressive disease despite initiation of treatment. Conclusion Misdiagnosis and inadvertent surgical intervention in cases of retinoblastoma in combination with delayed initiation of appropriate treatment is associated with poor prognosis. High index of suspicion for retinoblastoma is needed to avoid misdiagnosis and mismanagement.

To study the concordance between intra-operative rapid frozen section and permanent section diagnoses of surgical margins following wide surgical excisional biopsy of malignant eyelid tumors. This is a retrospective study of 120 cases and 429 frozen section slides. Of 120 cases, 75 (63%) had sebaceous gland carcinoma, 34 (28%) had basal cell carcinoma, and 11 (9%) had squamous cell carcinoma. All cases with these malignant eyelid tumors underwent wide surgical excisional biopsy under frozen section control of surgical margins. A total of 429 frozen section slides were reviewed for rapid frozen section diagnosis. Eyelid reconstruction was performed in all cases after clearance was obtained by rapid frozen section diagnosis of surgical margins as negative for tumor infiltration. Permanent section diagnosis of surgical margins was positive for tumor infiltration in 5 (1%) slides, which were reported as negative on rapid frozen section diagnosis of surgical margins, and was negative for tumor infiltration in 3 (

Purpose To discuss the importance of routine ophthalmic examination of parents and siblings of retinoblastoma (RB) patients. Methods Prospective nonrandomized observational/interventional case series of consecutive families of 131 RB patients. Results Routine ophthalmic examination of families (parents and siblings) of 131 consecutive newly diagnosed RB patients, including 262 parents and 23 siblings, revealed spontaneously regressed RB in at least 1 parent of 10 (8%) patients and active RB in at least 1 sibling of 3 (2%) patients. Of the 10 parents with spontaneously regressed RB, the lesions were unilateral (n = 7) or bilateral (n = 3). The regression patterns (n = 13) were comparable with postirradiation regression patterns Type 1 (n = 3), Type 2 (n = 2), Type 3 (n = 2), and Type 4 (n = 3), and spontaneous phthisis bulbi (n = 3). Fundus screening of siblings revealed active RB in at least 1 sibling of 3 (2%) patients. Of these 3 siblings, 2 had unilateral and 1 had bilateral disease. The mean age at detection of RB was 15 months (median, 6 months; range, 2-36 months). The disease was unilateral in 2 and bilateral in 1 patient. Based on International Classification of Intraocular Retinoblastoma, the tumors (n = 4) were classified as Group A (n = 2) and Group B (n = 2). Conclusion Routine fundus screening of siblings allows for early detection of RB in otherwise asymptomatic children. Detection of spontaneously regressed RB in parents may act as a surrogate marker for germline RB1 mutation and is helpful in genetic counseling.

Purpose: To describe the clinical features, treatment, and outcome of retinoblastoma in adults. Methods: Retrospective case series. Results: The mean age at initial presentation of retinoblastoma was 30 years (median, 26 years; range, 22–48 years). There were four males and four females, and all manifested unilateral retinoblastoma. The mean duration of symptoms was 22 months (median, 12 months; range, 1–100 months). Six patients had intraocular retinoblastoma, and 2 had secondary orbital involvement. The eyes with intraocular retinoblastoma were classified according to the International Classification of Retinoblastoma as Group D (n = 3) or Group E (n = 3). The primary treatment for intraocular retinoblastoma (n = 6) included systemic chemotherapy (n = 1), external beam radiotherapy (n = 2), and enucleation (n = 3). Secondary treatment for tumor recurrence included enucleation (n = 2), and combination of intraarterial chemotherapy, intravitreal chemotherapy, and plaque radiotherapy (n = 1). The eyes with orbital extension of retinoblastoma were classified according to the International Retinoblastoma Staging System as Stage 3a (n = 2). The primary treatment for those with orbital extension of retinoblastoma included multimodality treatment (combination of systemic chemotherapy, orbital exenteration, and external beam radiotherapy). Systemic metastasis and related death occurred in one case. Conclusion: Retinoblastoma in adults is uncommon. Active tumor in this age group is usually advanced, necessitating enucleation and/or orbital exenteration.

This study aims to describe the clinicopathological features and outcomes of patients who underwent orbital exenteration at a tertiary eye care center in south India. Retrospective chart reviews were performed on all patients undergoing orbital exenteration from January 1999 to December 2012. Parameters recorded include demographic data, clinical presentations, past medical or surgical interventions, exenteration notes, histopathological diagnosis, adjunctive treatment, follow-up examination findings, recurrences, complications, and their management. Orbital exenteration was performed on 119 orbits of 119 patients over a 14-year period. The mean age was 48.9 years (range 1-82 years). The indications were malignancies in 90.7 % (108/119), while 9.3 % (11/119) of cases were exenterated for non-malignant indications. Among the malignancies, the commonest tissue of origin was conjunctiva, noted in 45.4 % (49/108), followed by eyelids (25.9 %, 28/108), orbit (19.4 %, 21/108), and intraocular tissues (9.3 %, 10/108). The commonest malignancies noted in this series were an extensive ocular surface squamous neoplasia (OSSN) (44.4 %, 48/108) and sebaceous gland carcinoma of eyelids with an orbital involvement (18.5 %, 20/108). Extensive fungal granuloma (mucormycosis-4, aspergillosis-4) was the commonest (81.8 %, 9/11) indication among the non-malignant group. All complications were wound related, and none had a recurrence at a mean follow-up of 12.8 months. Extensive orbital involvement with OSSN and SGC were the commonest indications for exenteration. There is a need of creating awareness among general ophthalmologists in specific geographical regions regarding early diagnosis, standardized protocols of management, and appropriate referral.

A retrospective, nonrandomized, interventional case series of 8100 patients with uveal melanoma were evaluated for melanoma-related metastasis based on patient race. The patient race was Caucasian (n=7918, 98%), Hispanic (n=105, 1%), Asian (n=44

Purpose To describe and compare the clinical presentation, treatment outcomes, and histopathologic features of ocular surface squamous neoplasia (OSSN) based on human immunodeficiency virus (HIV) status. Design Case-control study. Participants A total of 200 patients with OSSN, of whom 83 (41%) had positive results for HIV and were classified as cases and 117 (59%) had negative results for HIV and were classified as controls. Methods Enzyme-linked immunosorbent assay for HIV, conjuntival excision biopsy, extended enucleation, orbital exenteration. Main Outcome Measures Clinical features, treatment outcomes, and histopathologic characteristics. Results The mean age at presentation of OSSN in both cases and controls was 40 years (median, 40 years; range, 13–65 years) and in controls was 40 years (median, 38 years; range, 15–80 years). On comparison of cases versus controls with OSSN, HIV-positive individuals had larger (12 vs. 8 mm; P P = 0.041) tumors, with a higher incidence of corneal (60% vs. 40%; P = 0.007), scleral (19% vs. 9%; P = 0.044), and orbital (13% vs. 3%; P = 0.019) invasion and a higher need for extended enucleation or exenteration (27% vs. 11%; P P = 0.13), need for lamellar sclerectomy (13% vs. 8%; P = 0.29), and tumor recurrence after primary treatment (30% vs. 20%; P = 0.12) was higher in HIV-positive cases compared with HIV-negative controls. However, these features were not statistically significant. Based on American Joint Committee on Cancer classification, T1 tumor was more common in controls (13% in cases vs. 35% in controls; P = 0.0009), and T4 tumor was more common in cases (13% in cases vs. 4% in controls; P = 0.019). None of the patients demonstrated systemic metastases or died of disease during a mean follow-up period of 10 months (median, 4 months; range, Conclusions Ocular surface squamous neoplasia in HIV-positive individuals is aggressive with larger and thicker tumors and with higher incidence of corneal, scleral, and orbital invasion. These patients are associated with poor ocular prognosis with higher need for extended enucleation, exenteration, or both.

Leiomyosarcoma is a common soft tissue tumor in the body. However, ocular leiomyosarcoma is rather uncommon. Herein, we describe the clinical and histopathological features of two cases of conjunctival leiomyosarcoma. There have only been three previously documented cases of conjunctival leiomyosarcoma.A 34-year-old male presented with a 2-year history of a whitish mass in the right eye. He underwent an incisional biopsy of the mass, which supported the diagnosis of leiomyosarcoma on histopathological examination. Computed tomography showed orbital extension of the mass, following which he underwent an eyelid sparing orbital exenteration of the right side. The second case was that of a 39-year-old male, who had a history of a whitish limbal mass, which had been previously excised elsewhere. The pre-operative clinical photographs and histopathology slides of the excised mass were reviewed. A histopathological diagnosis of conjunctival leiomyosarcoma was established and due to base positivity, he was treated with plaque radiotherapy. Both the cases showed no tumor recurrence or systemic metastasis at one-year follow-up.Primary conjunctival leiomyosarcoma is uncommon. Appropriate treatment of the tumor is associated with good prognosis.

Purpose To identify the clinical features predictive of high-risk retinoblastoma on histopathology. Design Case-control study. Participants A total of 145 cases with histopathologic high-risk features of retinoblastoma and 258 controls without high-risk features. Methods Enucleation and adjuvant chemotherapy. Main Outcome Measures High-risk features on histopathology were defined as the presence of anterior chamber seeds, iris infiltration, ciliary body infiltration, massive (≥3 mm) choroidal invasion, postlaminar optic nerve invasion, invasion of optic nerve transection, combined nonmassive choroidal and prelaminar/laminar optic nerve invasion, or scleral/extrascleral infiltration. Results Of 403 patients who underwent primary enucleation for the treatment of retinoblastoma, 145 (36%) had high-risk features on histopathology (cases) and 258 (64%) had no high-risk features (controls). High-risk retinoblastoma occurred in 16% of (8/50) group D eyes and 39% of (137/353) group E eyes. The histopathologic high-risk features in these 145 patients included anterior chamber seeds (n = 25, 17%), iris infiltration (n = 12, 8%), ciliary body infiltration (n = 17, 12%), massive (≥3 mm) choroidal invasion (n = 69, 48%), postlaminar optic nerve invasion (n = 71, 49%), invasion of optic nerve transection (n = 3, 2%), combined choroidal and optic nerve invasion (n = 17, 12%), scleral infiltration (n = 20, 14%), and extrascleral involvement (n = 8, 6%). The mean number of high-risk features was 2 (median, 2; range, 1–7). The significant clinical features in cases versus controls included prolonged duration of symptoms of >6 months (21% vs. 7%; P P = 0.05), buphthalmos (16% vs. 7%; P = 0.005), secondary glaucoma (47% vs. 15%; P P P P = 0.05). On the basis of International Classification of Intraocular Retinoblastoma, group E tumor had a statistically significant higher incidence of high-risk retinoblastoma compared with controls (39% vs. 16%; P = 0.01). Multivariate analysis of clinical features at presentation that predicted high-risk features on histopathology included prolonged duration of symptoms of >6 months ( P = 0.008) and secondary glaucoma ( P = 0.021). Conclusions In this study, the clinical features at presentation predictive of high-risk features on histopathology included prolonged duration of symptoms of >6 months and secondary glaucoma. Globe-preserving methods of treatment should be used with caution in patients with these features.

Purpose To analyze the clinical features and prognosis of posterior uveal melanoma based on the American Joint Committee on Cancer (AJCC) (7th edition) tumor staging. Design Retrospective interventional case series. Participants A total of 7731 patients. Methods Uveal melanoma management. Main Outcome Measures Melanoma-related metastasis and death. Results Of 7731 patients with posterior uveal (ciliary body and choroidal) melanoma, the AJCC tumor staging was stage I in 2767 (36%), stage II in 3735 (48%), stage III in 1220 (16%), and stage IV in 9 ( P P P P P P P P P Conclusions Compared with uveal melanoma classified as AJCC stage I, the rate of metastasis/death was 3 times greater for stage II, 9 to 10 times greater for stage III, and further greater for stage IV. Early detection of posterior uveal melanoma, at a point when the tumor is small, can be lifesaving.

Uveal melanoma is the most common primary malignant tumor of the eye in adults, predominantly found in Caucasians. Local tumor control of uveal melanoma is excellent, yet this malignancy is associated with relatively high mortality secondary to metastasis. Various clinical, histopathological, cytogenetic features and gene expression features help in estimating the prognosis of uveal melanoma. The clinical features associated with poor prognosis in patients with uveal melanoma include older age at presentation, male gender, larger tumor basal diameter and thickness, ciliary body location, diffuse tumor configuration, association with ocular/oculodermal melanocytosis, extraocular tumor extension, and advanced tumor staging by American Joint Committee on Cancer classification. Histopathological features suggestive of poor prognosis include epithelioid cell type, high mitotic activity, higher values of mean diameter of ten largest nucleoli, higher microvascular density, extravascular matrix patterns, tumor-infiltrating lymphocytes, tumor-infiltrating macrophages, higher expression of insulin-like growth factor-1 receptor, and higher expression of human leukocyte antigen Class I and II. Monosomy 3, 1p loss, 6q loss, and 8q and those classified as Class II by gene expression are predictive of poor prognosis of uveal melanoma. In this review, we discuss the prognostic factors of uveal melanoma. A database search was performed on PubMed, using the terms "uvea," "iris," "ciliary body," "choroid," "melanoma," "uveal melanoma" and "prognosis," "metastasis," "genetic testing," "gene expression profiling." Relevant English language articles were extracted, reviewed, and referenced appropriately.

To evaluate outcomes of ocular surface squamous neoplasia (OSSN) based on American Joint Committee on Cancer (AJCC), 8th edition classification. Retrospective, non-randomized interventional case series of 127 patients (136 eyes) with OSSN. On the basis of the AJCC (eighth edition), OSSN was classified as per T category as Tis (n = 14, 10%), T1 (n = 0), T2 (n = 4, 3%), T3 (n = 113, 83%), and T4 (n = 5, 4%). The following parameters increased with increasing T category: mean age at presentation at 37 years for Tis, 43 years for T2, 46 years for T3, and 55 years for T4 (p = 0.04); mean tumor basal diameter of 4 mm for Tis, not applicable (na) for T1, 6 mm for T2, 7 mm for T3, 20 mm for T4 (p = 0.001); extent of clock hours of corneal involvement (0, na, 0, 4, 8; p = 0.02), and conjunctival involvement (1, na, 2, 3, 9; p = 0.0005); involvement of adjacent structures including fornix (0%, na, 0, 9, 80%; p

The eye like any other organ in the body can harbor tumors of different types. Given the diverse anatomy of the eye, each distinct structure within the eye can be the site for primary tumors; that is, those tumors arising from the native tissue or metastatic tumors: those that arise from a distant site in the body. With reference to the eye and adnexa, metastatic tumors have been documented in the orbit, the extraocular muscles, conjunctiva, subconjunctival tissue, iris, and the retina with the most common site being the choroid [1]. This chapter presents in detail the clinical presentation, diagnosis, imaging findings, and treatment of retinal and choroidal metastases.

Purpose To report a single-center experience with nonsurgical correction of epiblepharon using hyaluronic acid gel. Methods The medical records of consecutive patients with symptomatic epiblepharon treated over a 3-year period with hyaluronic acid gel injection were reviewed retrospectively. Hyaluronic acid gel was injected transcutaneously into the suborbicularis plane to obliterate the abnormal skin fold or evert the eyelid margin. Successful treatment was defined as eversion of the eyelid margin as assessed by lash-cornea touch. Results Ten eyelids of 8 patients (7 girls [88%]) underwent transcutaneous hyaluronic acid gel injection for correction of epiblepharon. Average age at presentation was 16.5 months (range, 1-72 months). Two patients had bilateral involvement. Of 10 eyelids, 8 had a distinct skin fold with a “valley” above it. Nine of 10 eyelids had lash-cornea touch in the primary gaze; 1 in downgaze. All 10 eyes had punctate corneal epitheliopathy on fluorescein staining. An average of 0.19 ml (range, 0.1–0.3 ml) of hyaluronic acid gel was injected per eyelid. After injection, 9 of 10 eyelids showed no lash-cornea touch in downgaze, and all 10 eyelids showed resolution of symptoms and epitheliopathy. Patients remained symptom-free for an average final follow-up of 19.1 months (range, 5-42 months). No procedure-related complications were noted. Conclusions In our small case series, transcutaneous hyaluronic acid gel injection into the lower eyelid effectively corrected symptomatic epiblepharon; the effect was long lasting.

Optic disc pit and disc coloboma are common congenital anomalies. Both have been known to be associated with an orbital cyst. We report a case of a 6 month-old child who presented with unilateral progressive proptosis. Imaging investigations revealed a well-defined, intraconal, orbital cyst located close to the posterior surface of the globe displacing the optic nerve laterally. The cyst was excised and histopathological examination showed the cyst to have a lining of glial tissue, a thin epithelial lining, over a layer of collagenous connective tissue matrix. We hypothesize that colobomatous disc abnormalities and optic disc pits are different points on the same disease spectrum and can be associated with orbital cysts. Furthermore, our case points to the possible role of imaging the optic nerve in all cases of optic disc anomalies.

Purpose: To evaluate epiretinal membrane (ERM) response after cryotherapy for retinal vasoproliferative tumors (VPTs). Method: Retrospective interventional case series. Results: Of 16 eyes with VPT and ERM, the tumor was classified as primary in 12 (75%) eyes or secondary in 4 (25%) eyes. The median patient age was 44 years (mean, 43 years; range, 9–70 years). The tumor was located in extramacular zone (n = 16, 100%) and inferotemporal quadrant (n = 12, 75%). The mean tumor base was 6 mm, and thickness was 3 mm. The ERM involved the macula in 12 (75%) eyes and extramacular zone in 4 (25%) eyes, with best-corrected visual acuity of 20/40 or better in 6 (38%) eyes. Associated features included cystoid macular edema (n = 8, 50%), subretinal fluid (n = 10, 63%), vitreous cells (n = 9, 56%), and vitreous hemorrhage (n = 7, 44%). Single-session cryotherapy (double freeze–thaw) to the VPT was performed in each case. Over mean follow-up of 68 months (median, 54 months; range, 8–252 months), tumor regression was documented in 16 (100%) cases, with ERM release in 10 (63%) cases. After ERM release, the foveal anatomy was normal in 12 (75%) eyes. Final visual acuity improved (n = 5, 31%), remained stable (n = 9, 56%), or worsened (n = 2, 13%). Posttreatment best-corrected visual acuity was 20/40 or better in 10 (63%) eyes. Conclusion: Cryotherapy is remarkably effective for VPT of 6 mm or less in basal dimension. After cryotherapy, VPT-related ERM spontaneously released in 63% of the cases, without the need for surgical intervention.

Purpose: To describe the imaging features of choroidal metastasis using enhanced depth imaging optical coherence tomography (EDI-OCT). Methods: This retrospective observational case series included 31 eyes with choroidal metastasis. Spectral domain EDI-OCT was performed using Heidelberg Spectralis HRA + OCT. The main outcome measures were imaging features by EDI-OCT. Results: Of 31 eyes with choroidal metastasis imaged with EDI-OCT, 14 (45%) eyes displayed image detail suitable for study. The metastasis originated from carcinoma of the breast (n = 7, 50%), lung (n = 5, 36%), pancreas (n = 1, 7%), and thyroid gland (n = 1, 7%). The mean tumor basal diameter was 6.4 mm, and mean thickness was 2.3 mm by B-scan ultrasonography. The tumor location was submacular in 6 (43%) eyes and extramacular in 8 (57%) eyes. By EDI-OCT, the mean tumor thickness was 987 μm. The most salient EDI-OCT features of the metastasis included anterior compression/obliteration of the overlying choriocapillaris (n = 13, 93%), an irregular (lumpy bumpy) anterior contour (n = 9, 64%), and posterior shadowing (n = 12, 86%). Overlying retinal pigment epithelial abnormalities were noted (n = 11, 78%). Outer retinal features included structural loss of the interdigitation of the cone outer segment tips (n = 9, 64%), the ellipsoid portion of photoreceptors (n = 8, 57%), external limiting membrane (n = 4, 29%), outer nuclear layer (n = 1, 7%), and outer plexiform layer (n = 1, 7%). The inner retinal layers (inner nuclear layer to nerve fiber layer) were normal. Subretinal fluid (n = 11, 79%), subretinal lipofuscin pigment (n = 1, 7%), and intraretinal edema (n = 2, 14%) were identified. Conclusion: The EDI-OCT of choroidal metastasis shows a characteristic lumpy bumpy anterior tumor surface and outer retinal layer disruption with preservation of inner retinal layers.

A patient with choroidal haemangioma in the right eye developed photodynamic therapy (PDT)-induced acute exudative maculopathy within a week of being subjected to PDT (for treating the choroidal haemangioma). The condition was managed with an intravitreal injection of bevacizumab and responded well to therapy.

A 1-day-old child was brought to the clinic for evaluation of enlarged right eye (OD). On examination, OD showed buphthalmos with diffuse scleral melanocytosis, fleshy blackish-brown extrascleral mass with corneal extension, and secondary glaucoma. Anterior segment evaluation revealed darkly pigmented iris and fundus evaluation OD revealed a darkly pigmented choroidal lesion. The left eye was within normal limits. A clinical diagnosis of choroidal melanocytoma with ocular melanocytosis was made. Enucleation OD followed by orbital implant was performed. Histopathology showed features of diffuse ocular melanocytosis involving limbus, iris, ciliary body, choroid, sclera, optic nerve head, optic nerve sheath, along with choroidal melanocytoma with extrascleral tumour extension. We presume that choroidal melanocytoma may have arisen from ocular melanocytosis.