Intraocular medulloepithelioma: an unusual and challenging entity in paediatric population.

Intraocular medulloepithelioma is a rare, congenital tumour of the non-pigmented ciliary epithelium. It most frequently arises from the ciliary body but can also have its origin from the retina, iris and optic nerve. The age when lesion first appears is typically around 2-10 years. Nearly 50-60% of patients having this lesion may also have secondary features such as cataract and neovascular glaucoma. Those with extrascleral medulloepithelioma are at risk for metastasis. Systemic correlation of the tumour with pleuropulmonary blastoma/DICER1 gene is reported in the literature. Here, we report a case of a 15 years old boy with one year history of right eye proptosis and painful red right eye along with decreased vision for one week. He was assessed and operated for cataract elsewhere three years back. The ophthalmology team managed him for endophthalmitis with intravenous antibiotics, followed by 2 sessions of cryotherapy and finally an enucleation of right eye was performed due to severe pain and no vision in the involved eye. His left eye, general physical examination and systemic evaluation were normal. Histopathology revealed the diagnosis of 'malignant teratoid medulloepithelioma'. Therefore, evaluation of systemic associations for DICER1 gene mutations was performed by the oncology team. For high risk feature of scleral invasion on histopathology, he was treated with chemotherapy. Since the tumour is of rare occurrence; an international expert team with vast research experience in PPB/DICER1 associated tumours was also contacted. He was registered in International PPB/DICER1 registry where a detailed central radiology and pathology review was performed. Genetic counseling and surveillance plan was also suggested by the international registry.

Idiopathic Choroidal Neovascularization in Pregnancy: A Case Report.

Choroidal neovascularization (CNV) is the abnormal growth of vessels from the choroidal vasculature to the neurosensory retina through the Bruch's membrane and is usually associated with "wet" age-related macular degeneration (AMD). Other causes include myopia, traumatic rupture of the choroid, multifocal choroiditis, and histoplasmosis. CNV is a major cause of visual loss and treatment is aimed at halting progression and stabilizing vision. Intravitreal anti-vascular endothelial growth factor (IVT anti-VEGF) injection is the treatment of choice for CNV regardless of etiology. However, its use in pregnancy is debatable, due to its mechanism of action and lack of evidence of safety in pregnancy. Herein, we report a 27-year-old pregnant female, who complained of blurred and decreased vision in her left eye for two weeks. On examination, her unaided vision was 6/6 in her right eye and 6/18 partial in her left eye with no further improvement. Based on history, examination, and investigations she was diagnosed as a case of idiopathic CNV in pregnancy, being only the sixth reported case worldwide. Citing the risk of possible fetal adverse effects, the patient did not consent to the treatment despite extensive counseling. She was advised to follow up regularly and to receive IVT anti-VEGF injections immediately after delivery. A literature review was therefore undertaken to broaden our understanding of the treatment protocols and outcomes of using IVT anti-VEGF in pregnancy. This helped us to develop an understanding of the possible relative safety of such a treatment when individually tailored with a multi-disciplinary approach.

Efficacy Of Diode Laser In The Management Of Glaucoma Following Intravitreal Silicone Oil Injection In Complicated Retinal Detachment.

BACKGROUND: Intravitreal silicone oil injection has been used to manage complicated retinal detachment. This study aims to analyse the efficacy of diode laser (TSCPC) in the management of secondary glaucoma following intravitreal silicone oil injection in complex retinal detachment surgery. METHODS: The medical records of 58 eyes of 58 patients retrospectively were reviewed. Patients who underwent TSCPC for the management of secondary glaucoma following intravitreal silicone oil injection in complex retinal detachment were included. Procedure performed under local anaesthesia. The laser power was set at 1500-2200 mW for 2 sec. At each follow-up visit, patients went under charting of their IOP measurements with anterior segment and fundus examination. Post procedural follow up visits were carried out as; at 1 week, 1st, 3rdand 6thmonths. The efficacy was measured as reduction in IOP up to ≤20 mmHg without further needs for medications at 6 months. RESULTS: The IOP was reduced up to 19.26±10.15 mmHg. Comparisons of IOP at each interval with pre-treatment showed significant difference with p<0.0001. The efficacy of procedure was achieved in 77.6% patients. Age, gender and pre-operative IOP showed significant association with p<0.05. CONCLUSIONS: Diode laser TSCPC was effective and safe in reducing IOP. The IOP decreases gradually over six month follow up. The procedure is simple and cost-effective yet reasonably afforded by patients in low resources population. In spite of the retained intravitreal silicon oil patients with failed medical treatment for uncontrolled IOP can be treated with TSCPS.

Corneal approach 20 Guage vitrectomy system for the management of congenital cataract.

OBJECTIVE: To evaluate the efficacy and complications of 20 gauge vitrectomy via corneal approach for the management of congenital cataract. METHOD: We performed anterior capsular vitreorhexis, lens matter aspiration (LMA), primary posterior vitrectorhexis and anterior vitrectomy via corneal approach using 20 gauge vitrectomy system in children younger than two years of age with congenital cataract between January 2014 to December 2014. The intra and postoperative complications were observed. RESULTS: Twenty nine eyes of 21 children were included in this study. Congenital cataract surgery using 20 gauge vitrectomy system via corneal approach did not reveal any intra operative complication. Post operatively all children were able to freely open their operated eyes. Conjunctival congestion at the incision site in four eyes and mild anterior chamber reaction in 8 eyes were seen on 1(st) daywhich resolved at one week follow up. Other major post operative complications such as inflammatory membrane, irregular pupil, posterior/anterior syneache and opacification of visual axis were not seen during follow up period. CONCLUSION: The 20-gauge vitrectomy system via corneal approach is easy to perform, is less time consuming, safe and effective for the management of congenital cataract in younger children.