Autosomal Recessive Bestrophinopathy: Clinical and Genetic Characteristics of Twenty-Four Cases.

BACKGROUND: To describe ocular manifestations, imaging characteristics, and genetic test results of autosomal recessive bestrophinopathy (ARB). The study design is an observational case series. METHODS: Forty-eight eyes of 24 patients diagnosed with ARB underwent complete ophthalmic examinations including refraction, anterior and posterior segment examination, enhanced depth imaging optical coherence tomography (EDI-OCT), fluorescein angiography (FA), electroretinography (ERG), and electrooculography (EOG). Optical coherence tomography angiography (OCTA) and BEST1 gene sequencing were performed in selected patients. RESULTS: The age at onset was 4-35 years (mean: 18.6 years). The male-to-female ratio was 0.45. All patients were hyperopic, except one with less than one diopter myopia. EOG was abnormal in 18 cases with near-normal ERGs. Six patients did not undergo EOG due to their young age. Eighteen patients (75%) had a thick choroid on EDI-OCT, of which three had advanced angle-closure glaucoma, 15 patients were hyperopic, and eight of them had more than four diopters hyperopia in both eyes. Macular retinoschisis was observed in 46 eyes of 23 patients (95%) with cysts mostly located in the inner nuclear layer (INL) to the outer nuclear layer (ONL). Of the 18 patients who underwent FA, mild peripheral leakage was seen in eight eyes of four patients (22%). Subfoveal choroidal neovascularization (CNV) was seen in three eyes of two patients (6%) that responded well to intravitreal bevacizumab (IVB). Seven mutations of the bestrophin-1 (BEST1) gene were found in this study; however, only two of them (p.Gly34 = and p.Leu319Pro) had been previously reported as the cause of ARB based on ClinVar and other literature studies. CONCLUSIONS: ARB can be presented with a wide spectrum of ocular abnormalities that may not be easily diagnosed. Pachychoroid can occur alongside retinal schisis and may be the underlying cause of angle-closure glaucoma in ARB. Our study also expands the pathogenic mutation spectrum of the BEST1 gene associated with ARB.

Modeling a Telemedicine Screening Program for Diabetic Retinopathy in Iran and Implementing a Pilot Project in Tehran Suburb.

PURPOSE: To model a community-based telescreening program for diabetic retinopathy (DR) in Iran and to implement a pilot project at the Iranian Diabetes Society (IDS) branch in a Tehran suburb. METHODS: In this mixed model study, a web application called the "Iranian Retinopathy Teleophthalmology Screening (IRTOS)" was launched. The educational course for DR screening was established for general practitioners (GPs). Registered patients in IDS branch were recalled for fundus photography; images were transferred to the reading center via IRTOS to be graded by GPs, and patients were informed about the results via mobile messaging. All images were independently reviewed by a retina specialist as the gold standard. Patients who required further assessment were referred to an eye hospital. RESULTS: Overall, 604 subjects with diabetes were screened; of these, 50% required referral. The sensitivity and specificity for diagnosis of any stage of DR by trained GPs were 82.8% and 86.2%, respectively, in comparison to the gold standard. The corresponding values for detecting any stage of diabetic macular edema (DME) were 63.5% and 96.6%, respectively. CONCLUSIONS: Telescreening was an effective method for detecting DR in a Tehran suburb. This screening model demonstrated its capacity for promoting diabetic eye care services at the national level. However, the sensitivity for detecting DME needs to be improved by modifying the referral pathway and promoting the skill of GPs.

Intravitreal Injection of Anti-vascular Endothelial Growth Factor Agents for Ocular Vascular Diseases: Clinical Practice Guideline.

PURPOSE: To provide the clinical recommendations for the administration of intravitreal anti-vascular endothelial growth factor (VEGF) drugs especially bavacizumab for ocular vascular diseases including diabetic macular edema, neovascular age-related macular degeneration, myopic choroidal neovascularization, retinal vein occlusion and central serous chorioretinopathy. METHODS: Twenty clinical questions were developed by the guideline technical committee. Relevant websites and databases were searched to find out the pertinent clinical practice guidelines to answer the questions. The technical committee provided possible answers (scenarios) according to the available evidences for each question. All scenarios along with their levels of evidence and the supported articles were sent to the experts for external review. If the experts did not agree on any of the scenarios for one particular clinical question, the technical committee reviewed all scenarios and their pertinent evidences and made the necessary decision. After that, the experts were asked to score them again. All confirmed scenarios were gathered as the final recommendations. RESULTS: All the experts agreed on at least one of the scenarios. The technical committee extracted the agreed scenario for each clinical question as the final recommendation. Finally, 56 recommendations were developed for the procedure of intravitreal anti-VEGF injection and their applications in the management of ocular vascular diseases. CONCLUSION: The implementation of this guideline can standardize the management of the common ocular vascular diseases by intravitreal injection of anti-VEGF agents. It can lead to better policy-making and evidence-based clinical decision by ophthalmologists and optimal evidence based eye care for patients.

Two Doses of Intravitreal Ziv-Aflibercept versus Bevacizumab in Treatment of Diabetic Macular Edema: A Three-Armed, Double-Blind Randomized Trial.

PURPOSE: To compare the efficacy of 2 doses of intravitreal ziv-aflibercept (IVZ) with intravitreal bevacizumab (IVB) in the treatment of center-involved diabetic macular edema (DME) at 12 weeks. DESIGN: Three-armed, double-blind, randomized clinical trial. PARTICIPANTS: Eyes with center-involved DME. METHODS: In this trial, 123 eyes with DME were randomly assigned to 3 injections of 1.25 mg IVZ, 2.5 mg IVZ, and 1.25 mg IVB every 4 weeks. Complete ophthalmologic examination and central macular thickness (CMT) measurement by optical coherence tomography were performed every 4 weeks up to 12 weeks. MAIN OUTCOME MEASURES: Change in best-corrected visual acuity (BCVA) at 12 weeks. RESULTS: Although no significant difference was evident between the 2 ziv-aflibercept groups at 12 weeks, the BCVA change was significantly better in the ziv-aflibercept 1.25 mg group than in the IVB group at the 12-week visit (P = 0.021). In regard to CMT changes, there was no significant difference between the 2 ziv-aflibercept groups; however, a significantly greater reduction in CMT was observed in the ziv-aflibercept 2.5 mg group compared with the IVB group at 12 weeks (P = 0.037). Subgroup analysis disclosed no difference in BCVA outcomes at 12 weeks among the groups in the eyes with baseline BCVA ≥20/50. In the eyes with baseline BCVA <20/50, the improvement was significantly better at 12 weeks in the ziv-aflibercept 1.25 mg group compared with the IVB group (P = 0.011). CONCLUSIONS: The 12-week results of this trial disclosed that both 1.25 mg and 2.5 mg doses of IVZ and IVB demonstrated BCVA improvement over baseline in the treatment of center-involved DME. However, a stronger effect of IVZ compared with IVB in terms of both visual acuity improvement and macular thickness reduction was detected in the eyes with initial BCVA <20/50. Longer-term efficacy and safety data will be needed to understand the role for this drug in practice.

Diabetic Retinopathy Clinical Practice Guidelines: Customized for Iranian Population.

PURPOSE: To customize clinical practice guidelines (CPGs) for management of diabetic retinopathy (DR) in the Iranian population. METHODS: Three DR CPGs (The Royal College of Ophthalmologists 2013, American Academy of Ophthalmology [Preferred Practice Pattern 2012], and Australian Diabetes Society 2008) were selected from the literature using the AGREE tool. Clinical questions were designed and summarized into four tables by the customization team. The components of the clinical questions along with pertinent recommendations extracted from the above-mentioned CPGs; details of the supporting articles and their levels of evidence; clinical recommendations considering clinical benefits, cost and side effects; and revised recommendations based on customization capability (applicability, acceptability, external validity) were recorded in 4 tables, respectively. Customized recommendations were sent to the faculty members of all universities across the country to score the recommendations from 1 to 9. RESULTS: Agreed recommendations were accepted as the final recommendations while the non-agreed ones were approved after revision. Eventually, 29 customized recommendations under three major categories consisting of screening, diagnosis and treatment of DR were developed along with their sources and levels of evidence. CONCLUSION: This customized CPGs for management of DR can be used to standardize the referral pathway, diagnosis and treatment of patients with diabetic retinopathy.

Systemic oxygen therapy versus oral enalapril for treatment of diabetic macular ischemia: a randomized controlled trial.

The purpose of this study was to evaluate the structural and functional effects of systemic oxygen therapy and enalapril in patients with diabetic macular ischemia (DMI). This randomized clinical trial consisted of 105 eyes with DMI divided into three groups. Group I received systemic oxygen by face mask at a flow rate of 10 L/min; Group II received 5 mg enalapril daily; and Group III received placebo tablets for 3 months. Best-corrected visual acuity (BCVA), central macular thickness (CMT) measured by optical coherence tomography (OCT), extent of foveal avascular zone (FAZ) on fluorescein angiograms, and electroretinograms (ERG) were obtained at baseline and after 3 and 6 months. Overall, 102 patients completed the study. Baseline characteristics were not significantly different among groups. Significant improvement in BCVA and decrease in CMT and FAZ occurred at months 3 and 6 in oxygen group compared to deterioration in enalapril and control groups (All P values <0.001). ERG parameters were significantly better in oxygen group compared to enalapril group at months 3 and 6 and better than those in control group at month 3. Normobaric oxygen therapy for 3 months in DMI decreased CMT and FAZ and improved BCVA and ERG parameters. Enalapril did not show any favorable effect.

Electroretinogram changes in the sound eye of subjects with unilateral necrotizing herpetic retinitis.

PURPOSE: To evaluate electroretinogram (ERG) changes in the contralateral normal appearing eye of patients with unilateral acute necrotizing herpetic retinitis (NHR). METHODS: This interventional case series includes subjects with acute unilateral NHR. All patients were treated with intravenous followed by oral acyclovir and systemic steroids. Main outcome measures were changes in a- and b-wave amplitudes of scotopic and photopic full-field ERG in the sound eye, 1 and 3 months after therapy as compared to baseline. Twenty normal subjects served as controls. RESULTS: Forty eyes of 20 patients including 12 male and 8 female subjects with mean age of 44.1±11.5 (range 22 to 66) years were studied. Twenty unaffected eyes were the subject of the current study. The retina in all of these eyes remained intact during the course of the study. In the sound eyes, mean b-wave amplitude of the maximal combined response ERG before initiation of treatment was 229.5±38.8 microvolts which increased to 356.1±34.0 (P<0.001) and 365.8±32.7 (P<0.001) microvolts 1 and 3 months after treatment, respectively. Corresponding figures for b-wave amplitudes of the cone response ERG were 24.9±6.0, 47.0±12.9 (P<0.001) and 52.8±12.7 (P<0.001) microvolts, respectively. Visual acuity of all sound eyes remained unchanged throughout the study. CONCLUSION: Despite normal retinal appearance and intact visual acuity in the sound eyes of patients with NHR, electrophysiological changes were observed. Prompt diagnosis and management of NHR and continuation of medication for 3 months may reverse subclinical ERG changes and reduce the risk of progression to overt clinical disease.

Rate of Post-traumatic Endophthalmitis with or without Injection of Balanced Salt Solution.

PURPOSE: In a study complementing a previous multicenter randomized clinical trial on prophylactic injection of intraocular antibiotics during primary repair of penetrating eye injuries (PEIs), we sought to determine whether needle entrance and injection of balanced salt solution (BSS), per se, could increase the rate of acute post-traumatic bacterial endophthalmitis (APBE). METHODS: Patients randomized to the BSS injection arm (n=167) of the Traumatic Endophthalmitis Trial, and eligible patients who had refused enrollment and received no intraocular injections during primary repair (n=111) were compared for the development of APBE. RESULTS: APBE occurred in 8 of 167 (4.8%) eyes in the BSS group and in 5 of 111 (4.5%) eyes in the non-injection group (P=0.91). Retained intraocular foreign bodies were present in 46 eyes including 25 (15%) eyes in the BSS injection group and 21 (18.9%) eyes in the non-injection group (P=0.38). Logistic regression analysis showed no significant difference between BSS injected and non-injected eyes in terms of APBE (P=0.69). However, the presence of intraocular foreign bodies was strongly associated with the risk of endophthalmitis (P<0.001, OR=14.1, 95% CI: 4.1-48.5). CONCLUSION: Needle entrance and intraocular injection of BSS during primary repair of PEIs does not increase the risk of APBE.

The six syndromes of the sixth cranial nerve.

The sixth cranial nerve runs a long course from the brainstem to the lateral rectus muscle. Based on the location of an abnormality, other neurologic structures may be involved with the pathology related to this nerve. Sixth nerve palsy is frequently due to a benign process with full recovery within weeks, yet caution is warranted as it may portend a serious neurologic process. Hence, early diagnosis is often critical for some conditions that present with sixth nerve palsy. This article outlines a simple clinical approach to sixth nerve palsy based on its anatomy.

Electroretinographic changes following retinal reattachment surgery.

PURPOSE: To explore functional visual recovery after retinal reattachment surgery employing full-field electroretinography (ffERG). METHODS: In this case series, scotopic and photopic ffERGs were compared 2 days before, and 1, 3 and 6 months after successful scleral buckling for total rhegmatogenous retinal detachment (RRD). Main outcome measures were changes in ERG a-and b-wave amplitudes postoperatively. RESULTS: Twenty eyes of 20 patients including 14 male and 6 female subjects with mean age of 34.7±8.2 (range, 23 to 50) years were enrolled. Preoperatively, mean a-wave amplitude in the maximal combined response was 27.5±11.7 mV which was increased to 110.7±41.9 (P<0.001), 175.7±53.1 (p<0.001) and 174.6±51.4 (P<0.001) mV at 1, 3 and 6 months, respectively. Mean preoperative a-wave amplitude of the cone ERG response was 2.1±0.8 mV, which was increased to 2.2±0.9 (P=0.03), 5.1±1.7 (P<0.001) and 5.3±1.6 (P<0.001) mV at 1, 3 and 6 months, respectively. Mean preoperative b-wave amplitude in the maximal combined response was 97.6±28.9 mV which was increased to 179.2±44.9 (P<0.001), 264.2±56.3 (P<0.001) and 267.8±54.2 (P<0.001) mV at 1, 3 and 6 months, respectively. Mean preoperative b-wave amplitude of the cone ERG response was 2.9±0.9 mV which was increased to 3±0.9 (P=0.32), 9.9±1.9 (P<0.001) and 9.8±1.9 (P<0.001) mV at 1, 3 and 6 months, respectively. CONCLUSION: After retinal reattachment surgery, photoreceptor and visual function show parallel improvement. The scotopic ERG response recovered faster than the photopic response. Incomplete recovery of ERG parameters indicates that photoreceptor cell damage in retinal detachment is not completely reversible.

The effect of fluorescein angiography on full-field electroretinography parameters.

PURPOSE: To investigate the effect of simultaneously performed fluorescein angiography (FA) on full-field electroretinography (ffERG) parameters. METHOD: Scotopic and photopic ffERG were performed immediately and 60 minutes after conventional FA in patients with retinal photoreceptor disorders; a-and b-wave amplitudes were compared between recordings obtained at the two time intervals in each patient. RESULTS: Ten eyes of five (3 male and 2 female) patients with mean age of 19.6±3.8 (range, 15-25) years were studied. Intravenous fluorescein administration caused an immediate reduction in ERG waves which was most prominent in rod and maximal combined responses. Mean a-wave amplitude in maximal combined response, rod response and cone response ERGs was 46.0±18.8, 8.0±7.0 and 5.1±2.0 mv immediately after FA which was increased to 79.0±30.0, 21.5± 22.5 and 6.5±2.4 mv 60 minutes afterwards, respectively (P<0.005 for all comparisons). Mean b-wave amplitude in the same order was 91.0±17.5, 47.7±17.2 and 17.3±14.7mv which was increased to 145.0±54.3, 91.8±48.1 and 20.0±17.7 mv respectively, 60 minutes after FA (P<0.005 for all comparisons). CONCLUSION: The amplitude of ERG a- and b-waves under scotopic and photopic conditions increased significantly one hour after FA. These changes may be explained by disappearance of phototoxic and bleaching effects of strong light exposure from the light source of the angiography machine and fluorescein molecule on retinal photoreceptors.

Intravitreal bevacizumab vs. sham treatment in acute branch retinal vein occlusion with macular edema: results at 3 months (Report 1).

BACKGROUND: The goal of this work is to compare the visual and anatomical (central macular thickness; CMT) outcomes of intravitreal bevacizumab (IVB) injections relative to sham treatment in eyes with acute (less than 3 months in duration) branch retinal vein occlusion (BRVO). METHODS: In a double-masked randomized clinical trial (RCT), patients with acute BRVO were randomly assigned to one of two treatment groups: IVB (two injections of 1.25 mg IVB 6 weeks apart) or sham treatment. Primary outcome measures included changes in best-corrected visual acuity (BCVA) and CMT in optical coherence tomography (OCT) during follow-up (FU) examinations. Any complications secondary to injections were considered secondary outcomes. FU results after 6 and 12 weeks are reported. RESULTS: Eighty-one eyes (43 OD) of 81 patients (47 females) were enrolled in the study. Forty-two patients were enrolled in the IVB group, and 39 patients were enrolled in the sham group. Visual acuity and CMT improved in the IVB group after week 6 (two Snellen lines and 262 µm, respectively) and week 12 (three Snellen lines and 287 µm, respectively). After week 6, visual improvements in the IVB group were significantly increased relative to that of the sham group. However, visual improvements at week 12 were not significantly different between the two groups (1.5 Snellen lines visual improvement in the sham group at week 12). CONCLUSIONS: In acute BRVO, two IVB injections resulted in significant improvement of vision and CMT at 6 weeks relative to the sham group. However, the visual improvements in the IVB group were not significantly different from those in the sham group at 12 weeks. IVB injections accelerate an initial improvement of visual acuity but do not have any significant effects on vision after 12 weeks.

Oxygen therapy for diabetic macular ischemia: a pilot study.

PURPOSE: To evaluate the structural and functional effects of systemic oxygen therapy in patients with diabetic macular ischemia. METHODS: This interventional case series was performed on 20 eyes from 13 consecutive diabetic patients with macular ischemia. For each patient, baseline optical coherence tomography and electroretinography were obtained before and after administration of 100% oxygen by face mask at a flow rate of 10 L/minute for 1 hour. Central macular thickness and b-wave amplitude were measured before and after oxygen therapy. RESULTS: The patients included 9 women and 4 men with mean (SD) age of 63.38 (7.34) years. Central macular thickness decreased from 358.55 ± 96.27 µm before to 326.55 ± 84.11 µm after the oxygen therapy (P < 0.001). A reduction of >10% was observed in 55% of the eyes. In dark-adapted combined response electroretinography, the amplitude of b-wave increased from 227.76 ± 105.66 µV before to 264.35 ± 128.48 µV after the oxygen therapy (P < 0.001), representing a 16% increase. More than a 10% increase in b-wave amplitude occurred in 60% of the eyes. CONCLUSION: Oxygen therapy seems to have beneficial effects on the structural and functional changes of the retina in patients with diabetic macular ischemia. It may decrease central macular thickness and improve electrophysiologic function of the retina.

Electroretinogram Changes following Silicone Oil Removal.

PURPOSE: To evaluate electroretinogram (ERG) changes after silicone oil removal. METHODS: Scotopic and photopic ERGs, and best-corrected visual acuity (BCVA) were checked before and shortly after silicone oil removal in eyes that had previously undergone vitrectomy and silicone oil injection for complex retinal detachment. Pre- and postoperative ERG a- and b-wave amplitudes were compared. RESULTS: Twenty-eight eyes of 28 patients including 20 male and 8 female subjects with mean age of 39.3 ± 0.06 (range, 12 to 85) years were studied. Mean interval from primary vitreoretinal surgery to silicone oil removal was 21.04 ± 0.52 (range, 7 to 39) months. Mean duration from silicone oil removal to second ERG was 13.04 ± 1.75 (range, 10 to 16) days. Before silicone oil removal, mean a-wave amplitudes in maximal combined response, rod response and cone response ERGs were 27.4 ± 19.9, 7.2 ± 4.5 and 5.5 ± 3.4 µv, respectively. These values increased to 48.8 ± 31.9, 15.1 ± 14.4 and 17.4 ± 22.2 µv, respectively after silicone oil removal (P < 0.001). Mean b-wave amplitudes in the same order, were 69.41 ± 51, 41.2 ± 30.4 and 25.1 ± 33.9 µv before silicone oil removal, increasing to 165.6 ± 102.5, 81.7 ± 53.7 and 44.7 ± 34.1 µv respectively, after silicone oil removal (P < 0.001). Mean BCVA significantly improved from 1.10 ± 0.34 at baseline to 1.02 ± 0.33 logMAR after silicone oil removal (P < 0.001). CONCLUSION: The amplitudes of ERG a- and b-waves under scotopic and photopic conditions increased significantly shortly after silicone oil removal. An increase in BCVA was also observed. These changes may be explained by the insulating effect of silicone oil on the retina.

Increased Latency of Visual Evoked Potentials in Healthy Women during Menstruation.

PURPOSE: To evaluate the latency of visual evoked potentials (VEPs) in healthy women during and after menstruation. METHODS: Pattern and flash VEPs were performed in 15 healthy women aged 18 to 25 years on the maximum bleeding day (luteal phase) and 7 days after the menstrual cycle (follicular phase). RESULTS: Mean latency was 119.6 msec on the maximum bleeding day and 100.8 msec one week after menstruation on pattern VEP (P < 0.001). Corresponding values for flash VEP were 124.5 msec and 112.7 msec, respectively (P < 0.001). CONCLUSION: Prolonged VEP latency on the maximum bleeding day indicates that high progesterone levels may have an inhibitory effect on optic nerve conduction velocity.

Childhood pars planitis; clinical features and outcomes.

PURPOSE: To evaluate the demographic and clinical features of childhood pars planitis, and to determine the therapeutic and visual outcomes of the disease. METHODS: Medical records of pediatric patients (less than 16 years of age at diagnosis) with pars planitis and at least 6 months of follow-up who were referred to Labbafinejad Medical Center, Tehran, Iran over a 22 year period were reviewed. RESULTS: Overall, 117 eyes of 61 patients including 51 (83.6%) male subjects were included. Mean age at the time of diagnosis was 7.8±3.2 (range, 3-16) years. Mean best corrected visual acuity (BCVA) was 0.88±0.76 logMAR at presentation which improved to 0.39±0.51 logMAR at final visit (P<0.001). Endotheliitis was present in 23 (19.6%) eyes and was significantly more prevalent in subjects younger than 9 years (P=0.025). Cataract formation (41.9%) and cystoid macular edema (19.7%) were the most prevalent complications. Univariate regression analysis showed that better baseline visual acuity (OR=0.38, 95%CI 0.21-0.70, P=0.002), age older than 5 years at disease onset (OR=0.36, 95%CI 0.14-0.9, P=0.029), absence of endotheliitis (OR=0.39, 95%CI 0.15-0.99, P=0.047) and female gender (OR=3.77, 95%CI 1.03-13.93, P=0.046) were significantly associated with final BCVA of 20/40 or better. CONCLUSION: Childhood pars planitis was much more common among male subjects. Endotheliitis may be a sign of inflammation spillover and is more prevalent in younger patients. Visual prognosis is favorable in most patients with appropriate treatment.

Effect of oral prednisolone on visual outcomes and complications after scleral buckling.

PURPOSE: To evaluate the effect of oral prednisolone on visual outcomes and complications of scleral buckling (SB) in phakic eyes with acute rhegmatogenous retinal detachment (RRD). METHODS: In a randomized double-blind placebo-controlled trial, patients with acute RRD underwent SB. The treatment group received 1 mg/kg oral prednisolone for 10 days while the control group received placebo. Outcome measures included visual acuity (VA), retinal redetachment (re-RD), cystoid macular edema (CME), proliferative vitreoretinopathy (PVR), and other possible postoperative complications. Outcome measures were compared between groups with McNemar test. RESULTS: Overall, 52 eyes of 52 subjects were enrolled into the study, which included 25 and 27 eyes in the treatment and placebo groups, respectively. Preoperatively, the extent and location of RD and rate of hypotony was comparable between the study groups. VA improvement (logMAR) was 0.75+/-0.61 overall: 0.85+/-0.62 in the treatment group versus 0.65+/-0.61 in the placebo group (p=0.36). Postoperative complications in the treatment and placebo groups included choroidal detachment, 3 versus 4 eyes (p=0.45), significant PVR, 1 versus 3 (p=0.33), and CME, 3 versus 5 eyes (p=0.39), respectively. CONCLUSIONS: Postoperative oral prednisolone does not seem to improve the visual outcomes and complications of scleral buckling in simple phakic RRD.

Outcomes of retinal detachment surgery in eyes with chorioretinal coloboma.

PURPOSE: To report the anatomical and functional outcomes of surgery for retinal detachment associated with chorioretinal colobomas. METHODS: In this retrospective interventional case series, 28 eyes of 28 patients (including 18 male subjects) who had undergone surgery for retinal detachment associated with chorioretinal colobomas were evaluated regarding the type of intervention, final visual acuity and anatomical outcomes, as well as complications. Cases with less than 3 months of follow-up were excluded. RESULTS: Primary surgery included vitrectomy in 25 (89.3%) and scleral buckling in 3 (10.7%) eyes. The internal tamponade used in eyes undergoing vitrectomy was silicone oil in 23 (92%) eyes and 20% sulfur hexafluoride (SF6) in 2 (8%) eyes. Silicone oil was removed in 11 eyes (45.8%). The mean number of operations per eye was 1.57±0.74, mean follow-up was 40±36 months, and the retina remained attached in 26 eyes (92.9%) at final follow-up. Mean preoperative visual acuity was 2.33±0.55 (range, 1.15-2.9) logMAR which significantly improved to 1.72±0.9 (range, 0.09-3.1) logMAR postoperatively (P < 0.001), however, final median visual acuity was counting fingers at 2 m. The most common complications were cataracts (100%) and ocular hypertension (46.4%). CONCLUSION: The most prevalent surgical procedure for treatment of retinal detachment associated with chorioretinal coloboma was pars plana vitrectomy and the most frequently used tamponade was silicone oil. Although anatomical success was satisfactory, functional outcomes were not encouraging which reflects the complexity of the condition and associated abnormalities.