Outcome of surgery in patients with giant retinal tear: 10 years experience.

PurposeTo present our experience with the surgery in retinal detachment (RD) associated with giant retinal tear (GRT) over 10 years in a tertiary referral hospital.Patients and MethodsIn this retrospective study, the charts of all patients with the diagnosis of RD associated with GRT who underwent surgery from 2005 to 2015 at Rassoul Akram Hospital were reviewed. Exclusion criteria were concomitant presence of diabetic retinopathy, and uveitis. All patients had to have at least 3 months of follow up. The success rate and factors associated with repeated surgery were determined.ResultsSixty two eyes of 61 patients including 51 males and 10 females were assessed. Proliferative vitreoretinopathy (PVR) was present in 14 (22.6%) of eyes. Pars plana vitrectomy (PPV) alone was performed in 44 eye (71.0%) and simultaneous vitrectomy and phacoemulsification surgery was performed in 18 eyes (29.0%). An encircling episcleral band was placed in 7 eyes (11.3%). Anatomic success after one vitrectomy procedure was achieved in 45 eyes (72.58%) and ultimately in 61 eyes (98.4%) at last follow up. Seventeen eyes needed repeated PPV due to redetachment associated with PVR in the follow up period. The rate of repeated PPV was significantly higher in eyes with PVR at baseline and surgery with encircling episcleral band. Trauma, extension of tear, age, and lens status had no significant effect on the rate of repeated PPV.ConclusionsOur study shows that the high surgical success can be achieved in patients with RD associated with GRT with single or multiple surgeries.

Genetic screening in Iranian patients with retinoblastoma.

PurposeThe most common intraocular tumor in childhood, retinoblastoma, is largely associated with mutations in the RB1 gene. In the most comprehensive RB1 screening in Iran, we evaluated the RB1 mutations in 106 patients with retinoblastoma, including 73 bilateral (heritable) and 33 unilateral (sporadic) cases.Patients and methodsMutations were identified using amplification refractory mutation system (ARMS) PCR and direct sequencing of the 27 coding exons of RB1 and multiplex ligation-dependent probe amplification (MLPA).Results and ConclusionWe found 33 (31%) and 64 (60%) patients with sporadic unilateral and bilateral retinoblastoma, respectively as well as 9 (8.5%) cases with hereditary bilateral retinoblastoma. In total, we identified 52 causative RB1 mutations in 106 patients (global mutation rate of 49%). Of the 52 patients, 48 (92%) had sporadic and familial bilateral and 4 (8%) had sporadic unilateral RB. Therefore, the detection rate of RB1 mutations was 66% (48/73) and 12% (4/33) in bilateral and unilateral cases, respectively. Mutations were classified as nonsense in 31 (60%), missense in 1 (2%), large deletion in 11 (21%), small deletion in the 7 novel (15%) and splice site mutation in 2 (4%) patients with RB. Of 31 nonsense mutations, 23 (74%) occurred in the 11 Arginine codons of the RB1. Seven mutations (13%) were novel, and 45 (87%) had been previously reported. Thirty-three mutations were single-base substitutions leading to 31 nonsense amino acid changes and 2 splice site mutations in introns 12 and 16 of RB1. The altered 3D model structures of the RB1 novel mutant proteins are also predicted in this study.

Scleral buckling surgery for rhegmatogenous retinal detachment with subretinal proliferation.

PURPOSE: To evaluate the outcome of scleral buckling surgery in patients with rhegmatogenous retinal detachment (RRD) with subretinal proliferation. METHODS: In this retrospective study, a chart review of all patients with RRD associated with subretinal proliferation who were primarily treated with scleral buckling procedure, from April 2007 to April 2014, was undertaken. Main outcome measures were anatomical retinal reattachment and visual acuity. RESULTS: Forty-four eyes of 43 patients including 24 males and 19 females with a mean age of 26.5±13.1 years were evaluated. Immediately after the surgery, retina was reattached in all eyes. However, five eyes (11.3%) needed additional surgery for retinal redetachment. Single surgery anatomical success rate was 88.7%. Four eyes (9.1%), needed pars plana vitrectomy for the treatment of redetachment associated with proliferative vitreoretinopathy and scleral buckle revision surgery was successfully performed in the other eye. Best corrected visual acuity improved from 1.5±0.9 logMAR before surgery to 1.1±0.7 logMAR after surgery (P<0.001). An improvement in BCVA of >2 lines was found in 23 eyes (52.2%) and worsening of best corrected visual acuity of >2 lines was observed in 2 eyes (4.5%). CONCLUSIONS: Scleral buckling surgery is highly successful in eyes with RRD associated with subretinal proliferation.

Retinoblastoma in Iran: outcomes in terms of patients' survival and globe survival.

BACKGROUND/AIMS: Retinoblastoma is a highly malignant eye tumour in children with different survival rates across the world. The aims of this study are to determine the globe and patient survival in children with retinoblastoma in a major referral centre in Iran. METHODS: 156 eyes of 105 consecutive patients with retinoblastoma were enrolled from 2001 to 2007. All demographic data, family history, presenting symptoms, duration of symptoms, ocular findings and treatment modalities that were used for the patients were collected. For patient survival, event was defined as death and for globe survival as enucleation. RESULTS: The mean age at diagnosis was 28.5 months (unilateral 27.4 months; bilateral 30 months). Five patients had a positive family history. Fifty-two per cent of the cases were unilateral, and 48% were bilateral. The most common presenting sign was leucocoria (64.8%) followed by strabismus (28.2%). Enucleation was done primarily for 75.9% of unilateral cases and 34.3% of bilateral cases. Secondary enucleation was necessary in 5.6% and 7.8% of unilateral and bilaterally involved eyes respectively. Sixty-nine (44.2%) of 156 eyes were salvaged by different globe preserving modalities (unilateral 18.5%; bilateral 57.9%). The Kaplan-Meier survival estimate for globe preservation according to International Classification of Retinoblastoma (ICRB) was 100% for group A eyes, 93.5% for group B, 86.7% for group C, 57.1% for group D and 0% for group E eyes. Kaplan-Meier estimates for patients survival were 100% at 1 year, 94.8% at 3 years and 83.1% at 5 years. CONCLUSION: Progress in methods of treatment, early detection of the disease and prompt referral to specialised centres have led to improved outcomes for patients with retinoblastoma in terms of globe and patients' survival rates even in developing countries.

Pseudohypopyon of orange pigment overlying a stable choroidal nevus.

PURPOSE: To report an unusual case of orange pigment pseudohypopyon overlying a choroidal nevus. METHODS: Observational case report. A 45-year-old man presented with best-corrected visual acuity of 20/25 and metamorphopsia in the right eye secondary to localized subfoveal fluid. The detachment displayed a peculiar appearance of layered orange pigment, like a pseudohypopyon in its inferior aspect. Closer inspection revealed an underlying choroidal nevus and no choroidal neovascular membrane. RESULTS: After 31 months of follow-up without treatment, the size of the nevus and visual acuity remained stable and metamorphopsia continued to resolve as the orange pigment and the subretinal fluid disappeared completely. CONCLUSION: An otherwise stable choroidal nevus can display overlying clumps of orange pigment and rarely massive accumulation of orange pigment in the form of a pseudohypopyon. Orange pigment pseudohypopyon can spontaneously resolve with preserved visual acuity.