How ‘eye’ helped in von Hippel-Lindau syndrome

Background: Von Hippel-Lindau syndrome is a rare autosomal dominantly inherited multisystemic oncologic syndrome, presenting predominantly with angiomatosis in embryologically similar neurologic tissue such as retina, cerebellum and adrenals. Retinal hemangioblastomas are the hallmark ophthalmic finding. In this case report, we describe the importance of timely diagnosis, thorough systemic examination and treatment of bilaterally asymmetrical retinal hemangioblastomas in a young adult male. Case presentation: A 31-year-old male presented with painless diminution of vision in both eyes, associated with eyestrain and headache. Multiple asymmetric retinal lesions and dilated feeder vessels were noted on ophthalmoscopic examination and confirmed by fluorescein angiography to be retinal hemangioblastomas. Comprehensive systemic examination revealed cerebellar hemangioblastomas and multiple pancreatic and renal cysts. Treatment of retinal lesions was done by combination therapy of argon laser photocoagulation and cryopexy, which lead to a good visual outcome. Subsequently, neurosurgical resection of cerebellar hemangioblastoma proved to be lifesaving for the patient. Conclusion: RHBs are the earliest, easiest and the most frequently detected manifestation of VHL. Identification of ocular manifestations play a pivotal role in early diagnosis and timely intervention in VHL syndrome, thereby significantly reducing associated morbidity and mortality. Therefore, an ophthalmologist’s role is crucial in the management of these potentially deadly tumours.

Argon laser photocoagulation for treatment of presumed trematode-induced granulomatous anterior uveitis in children

Background/AimsTo assess the safety and efficacy of argon laser photocoagulation as a new modality for the treatment of presumed trematode-induced granulomatous anterior uveitis (PTGAU) in children.MethodsForty-eight eyes of 48 children with PTGAU with pearl-like nodule(s) in the anterior chamber were included in this prospective non-randomised controlled clinical trial. The patients were divided into two groups: those in Group A (23 eyes) were treated with one session of argon laser applied to the anterior chamber nodules and those in Group B (25 eyes) received medical treatment in the form of topical steroid and cycloplegic eye drops with trans-septal triamcinolone injections. All cases were followed up for 3 months with measurement of visual acuity (VA), assessment of the anterior chamber reaction and measurement of the pearl-like nodule size.ResultsIn Group A, 22 eyes (95.65%) showed regression of the pearl-like nodules with resolution of the anterior chamber reaction (flare and cells) and improvement in visual acuity from 0.52±0.12 to 0.06±0.08 logMAR (p<0.001). Such improvement was maintained within the 3-month follow-up period. In Group B, 23 eyes (92%) showed initial regression of the granulomas, which was maintained in only 14 eyes (56%),with nine eyes experiencing recurrence after 3 months of follow-up.ConclusionArgon laser photocoagulation is a safe and effective novel treatment for PTGAU with pearl-like nodules in the anterior chamber in children. Larger studies with longer follow-up periods are needed to confirm these results.

Argon Laser Photocoagulation for Treatment of Presumed Trematode-induced Granulomatous Anterior Uveitis in Children

Background/Aims: To assess the safety and efficacy of argon laser photocoagulation as a new modality for the treatment of presumed trematode-induced granulomatous anterior uveitis (PTGAU) in children. Methods: Forty-eight eyes of 48 children with PTGAU with pearl-like nodule(s) in the anterior chamber were included in this prospective non-randomised controlled clinical trial. Patients were divided into two groups: group A (23 eyes) was treated with one session of argon laser applied to the anterior chamber nodules, and group B (25 eyes) received medical treatment in the form of topical steroid and cycloplegic eye drops with trans-septal triamcinolone injection.All cases were followed up for 3 monthswith measurement of VA, assessment of anterior chamber reaction, and size of the pearl-like nodules. Results: In group A, 22 eyes (95.65%) showed regression of the pearl-like nodules with resolution of the anterior chamber reaction (flare and cells) and improvement in visual acuity from 0.52 0.12 to 0.06 0.08logMAR (p value <0.001). Such improvement was maintained within the 3-month follow-up period. In group B, 23 eyes (92%) showed initial regression of the granulomas,whichwas maintained in only 14 eyes (56%),with 9 eyes experiencing recurrence after 3 months of follow-up. Conclusion: Argon laser photocoagulation is a safe and effective novel treatment for PTGAU with pearl-like nodules in the anterior chamber in children. Larger studies with longer follow-up periods are needed to confirm these results.

Current Management of Combined Traction/Rhegmatogenous Retinal Detachments

Due to proliferative diabetic retinopathy (PDR) and related complications, 25% of diabetic patients experience significant visual impairment. Panretinal argon laser photocoagulation (PRP) and the use of vascular endothelial growth factors (VEGF) in recent years have reduced the frequency of visual loss and tractional retinal detachment (TRD) and combined tractional/rhegmatogenous retinal detachment (CTRD). TRD and CTRD are the primary indications for vitreoretinal surgery related to PDR. TRD and CTRD which elevate macula less than 6 months, rapidly progressive and threatening macula are the other important indications to vitreoretinal surgery in diabetic retinopathy. CTRD, which was almost inoperable in previous years, now it takes place in the indications to vitreoretinal surgery by the development of new surgical equipment (vertical and horizontal scissors, light systems, diamond knives, trocar systems, etc.) and new surgical techniques (segmentation, visco-dissection, en-block dissection). Small calibered (23, 25, 27 Gauge) vitrectomy probes shortened the duration of vitreoretinal surgery in TRD and CTRD cases. Preoperative anti-VEGF treatment inhibits the fibrovascular tissue activation in this way facilitates and enables safe surgery. As long as the vitreoretinal surgery equipment and techniques improved, the functional and anatomical success in CTRD and TRD will increase.

Photocoagulation for Diabetic Macular Edema

The Early Treatment Diabetic Retinopathy Study (ETDRS) was a randomized clinical trial involving nearly four thousand diabetic patients with early proliferative retinopathy, moderate to severe nonproliferative retinopathy, and/or diabetic macular edema in each eye. This paper (ETDRS report number 1) describes the findings in the subgroup of eyes in the ETDRS that were identified as having mild to moderate nonproliferative diabetic retinopathy and macular edema. The analysis showed that immediate focal argon laser photocoagulation of “clinically significant” diabetic macular edema substantially reduced the risk of visual loss, increased the chance of visual improvement, decreased the frequency of persistent macular edema, and caused only minor visual field losses. The authors recommended immediate focal argon laser photocoagulation for all eyes with clinically significant macular edema and mild or moderate nonproliferative diabetic retinopathy, regardless of the level of visual acuity.

Argon Laser Photocoagulation for Macular Edema in Branch Vein Occlusion

The Branch Vein Occlusion Study (BVOS) was a randomized, incompletely masked, controlled clinical trial in patients with a branch retinal vein occlusion (BRVO) occurring 3 to 18 months earlier, with best corrected visual acuity of 20/40 or worse from macular edema. The study showed that argon laser grid photocoagulation could improve visual acuity in eyes with BRVO and macular edema reducing visual acuity to 20/40 or worse. After this study, grid photocoagulation of the macula became the standard of care for BRVO with macular edema with foveal involvement and capillary leakage with visual acuity of 20/40 or worse, and it remains the gold standard therapy for perfused macular edema in BRVO. Advantages are that it is proven, widely available, nonsurgical, and of relatively moderate cost.