Longitudinal analysis of the structural pattern of pseudophakic cystoid macular edema using multimodal imaging.

PURPOSE: To describe the relationship between pseudophakic cystoid macular edema (CME) progression and retinal structural changes observed by spectral-domain optical coherence tomography (SD-OCT) and fluorescein angiography. METHODS: Retrospective, consecutive, longitudinal imaging analysis of patients with acute pseudophakic cystoid macular edema. Two observers at two study centers evaluated all images and categorized the macular structural features based on the retinal layer involved with cystic changes and/or subretinal fluid. A time series of the structural pattern and visual acuity was constructed for 1-month intervals over a 6-month study period. RESULTS: Thirty nine patients presented with CME. The mean time since cataract extraction was 48 ±38 days (range 5-71). CME included inner nuclear layer (INL) cystic changes, outer plexiform layer (OPL) cystic changes, and subretinal fluid. Isolated INL changes were observed in four phakic fellow eyes. Isolated OPL morphology was not observed, and additional inner layers remained uninvolved. Resolution occurred rapidly following administration of periocular glucocorticoids, and typically demonstrated a reverse structural pattern or delayed subretinal fluid resolution. Isolated subretinal fluid or a combined INL and subretinal fluid structure indicated CME resolution. CONCLUSION: Acute and recurrent pseudophakic CME involves a directional progression of the retinal layer involved with cystic changes and a clinical course. CME begins with INL cystic changes, progresses to combined INL and OPL morphology, and may continue to involve subretinal fluid. The inner nuclear layer is the most frequently involved layer, and isolated INL cysts may occur in phakic fellow eyes. A three-layer structure, the presence of OPL morphology, or isolated INL early in the postoperative period indicates disease activity.

Proliferative ischemic retinopathy after arteriovenous malformation embolization in a child with hereditary hemorrhagic telangiectasia.

PURPOSE: To describe a case of hereditary hemorrhagic telangiectasia, presenting with multiple branch retinal artery occlusions, retinal ischemia, neovascularization, and vitreous hemorrhage after cerebral arteriovenous malformation embolization. METHODS: The authors report a 7-year-old patient with decreased vision in his left eye after embolization of a pineal arteriovenous malformation secondary to hereditary hemorrhagic telangiectasia. Ophthalmic evaluation, fundus photography, fluorescein angiography, spectral domain optical coherence tomography, electroretinogram, examination under anesthesia, and pars plana vitrectomy (PPV) were performed. RESULTS: Fundus examination of the left eye revealed extensive posterior segment ischemia, vascular tortuosity, and vitreous hemorrhage. Fluorescein angiography was remarkable for partial obstruction of retinal arteries, midperipheral nonperfusion, and associated leakage from multiple areas of neovascularization. Spectral domain optical coherence tomography was normal. Electroretinogram demonstrated decreased b-wave amplitude. The patient was subsequently treated with 25-gauge pars plana vitrectomy, panretinal endophotocoagulation, and intravitreal bevacizumab. Five weeks after surgery, best-corrected visual acuity had improved to 20/40, and examination showed resolution of vitreous hemorrhage and neovascularization. CONCLUSION: Retinal vascular abnormalities, posterior segment ischemia, and vitreous hemorrhage suggested a combination of retinal involvement of hereditary hemorrhagic telangiectasia complicated by nontarget embolization.

Anatomical and visual outcomes after two-port pars plana vitrectomy reoperation under silicone oil for epimacular membrane or recurrent retinal detachment.

PURPOSE: To describe the anatomical and visual outcomes in a series of patients undergoing two-port pars plana vitrectomy reoperation under silicone oil for recurrent retinal detachment (RD) due to proliferative vitreoretinopathy or epimacular membrane (EMM) after RD repair. METHODS: This study is a prospective, consecutive, interventional case series of patients presenting with recurrent RD or EMM under silicone oil. Two-port 25-gauge pars plana vitrectomy reoperation without an infusion port was performed in all cases. RESULTS: Thirty-nine patients were included. Reoperation pathology included recurrent RD with proliferative vitreoretinopathy (n = 33) and EMM alone (n = 6). The mean number of previous retinal surgeries was 2.4 ± 1.1 (range, 1-5). The mean overall follow-up was 24 ± 3.7 months. The mean visual acuity change from baseline at final follow-up was an improvement of 0.74 ± 0.63. Macular reattachment was achieved in 29 of 33 patients with RD, and EMMs were successfully removed in all patients. CONCLUSION: Two-port pars plana vitrectomy reoperation is an efficacious method for repair of consecutive RD due to proliferative vitreoretinopathy or EMM in patients with previous RD repair with silicone oil. Significant visual improvement with a low complication rate may be achieved in patients with advanced proliferative vitreoretinopathy or EMM under silicone oil.

Intraoperative grasp site correlation with morphologic changes in retinal nerve fiber layer after internal limiting membrane peeling.

BACKGROUND AND OBJECTIVE: Recent reports have demonstrated inner retinal changes after internal limiting membrane (ILM) peeling, but the mechanism responsible for these findings remains poorly understood. The purpose of this report is to establish a correlation between ILM removal and postoperative morphologic changes. PATIENTS AND METHODS: Prospective, observational case series of eight consecutive patients undergoing pars plana vitrectomy with ILM peeling for macular hole or epimacular membrane. Intraoperatively, all grasp sites were recorded and subsequently superimposed on postoperative infrared and spectral-domain optical coherence tomography (SD-OCT) images. Repeat examination and imaging were performed at regular postoperative intervals. RESULTS: Infrared fundus photography revealed well-defined, hyporeflective arcuate striations in all patients during the early postoperative period. These defects followed the course of axonal pathways from the grasp site to the optic nerve. SD-OCT images on all patients revealed early focal nerve fiber layer swelling directly corresponding to grasp sites, with eventual atrophy. CONCLUSION: A dynamic process takes place within the inner retina following surgical removal of ILM. Inadvertent surgical trauma induced by ILM forceps may be the mechanism responsible for nerve fiber layer morphologic changes after ILM peeling.

Foreign body response within postoperative perfluoro-N-octane for retinal detachment repair: clinical features, grading system, and histopathology.

PURPOSE: To describe the clinical and histopathologic characteristics of inflammatory deposits occurring within intermediate duration postoperative perfluoro-n-octane (PFO) for inferior retinal detachment repair. METHODS: Prospective interventional case series of consecutive patients with inferior retinal detachment treated with intermediate duration postoperative vitreous cavity PFO endotamponade were analyzed by ophthalmoscopy for the presence of inflammation and white deposits. Ten consecutive samples developing white deposits were analyzed microscopically. Clinical variables were analyzed. RESULTS: One hundred and eighty-one eyes of 181 patients were included (mean age = 52.4 ± 14 years; mean follow-up = 29.7 ± 14 months). Fifty of 181 patients (28%) developed a characteristic foreign body response with abundant white deposits within indwelling PFO. Ten consecutive samples analyzed histologically contained abundant macrophages, the absence of additional inflammatory cells, and intracytoplasmic optically lucent inclusions. Foreign body response was associated with longer duration of PFO (P = 0.003). Perfluoro-n-octane foreign body response was not associated with age (P = 0.136), ethnicity (P = 0.101), visual outcome (P = 0.157), or persistent intraocular pressure elevation (P = 0.381). CONCLUSION: A stereotypical foreign body response occurs in ∼ 30% of patients with postoperative vitreous cavity PFO and becomes clinically apparent at 7 days to 10 days after initial placement for rhegmatogenous retinal detachment repair. The response consists almost exclusively of macrophages and does not seem to be related to either long-term intraocular pressure elevation or visual outcome. The response may be related to the duration of indwelling PFO and may limit visualization of the retina during PFO removal.

Current management of Coats disease.

Since its original description in 1908, Coats disease has been recognized as an idiopathic cause of severe vision loss with a remarkable diversity in clinical presentation and morphology. Key clinical and imaging variables are helpful in differentiating Coats disease from life-threatening malignancies, and proper management revolves around a thorough knowledge of the differential diagnosis. Despite significant advancement in scientific understanding of the disease process and clinical spectrum, the underlying etiology remains obscure, and both primary and secondary forms are recognized. With the development of anti-VEGF therapy, vitreoretinal specialists have a new, effective adjunct to the clinical management of exudates, macular edema, and serous retinal detachment. We highlight the history, diagnostic challenges, evolving clinical spectrum, and current management of Coats disease.

Submacular choroidal varix simulating chorioretinal folds with metamorphopsia.

A novel case description of an isolated unilateral submacular choroidal varix simulating chorioretinal folds and inducing metamorphopsia in a 74-year-old phakic Caucasian man. Posterior segment examination revealed focal choroidal elevations corresponding to a deep, large choroidal vessel in the inferotemporal subparafoveal region. Fundus photography demonstrated a pigment epithelial detachment-like elevation inferior to the fovea, continuous with the course of a large choroidal vessel. Angiographic, indocyanine green (ICG), and spectral-domain optical coherence tomography (SD-OCT) imaging revealed an isolated unilateral submacular choroidal varix with direct connection to an inferior vortex vein. The diagnosis of submacular choroidal varix should be considered when the presence of metamorphopsia and corresponding choroidal elevations that remain unchanged through gaze direction or contact lens ophthalmoscopy occur. If present, angiographic, ICG, and SD-OCT imaging are recommended for proper evaluation of submacular choroidal varix that may enhance the characteristics of this lesion.

Subacute exogenous Rhizoctonia solani endophthalmitis following cataract extraction with intraocular lens implantation.

UNLABELLED: Seven weeks after uneventful cataract extraction with intraocular lens (IOL) implantation, a 64-year-old man presented to his cataract surgeon with decreased vision and photophobia. The subacute presentation with anterior uveitis prompted initial therapy with topical and periocular glucocorticoids. One month later, the patient presented to the vitreoretinal service with counting fingers visual acuity, prominent anterior chamber reaction, a 2.5 mm hypopyon, and inflammatory deposits over the IOL. Cultures grew Rhizoctonia solani. The inflammation was successfully treated with pars plana vitrectomy, IOL explantation, and intravitreal voriconazole. Rhizoctonia should be included in the differential diagnosis of subacute exogenous endophthalmitis, especially in the context of fibrillar white IOL plaques. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.

Arcuate retinotomy for the repair of large macular holes.

BACKGROUND AND OBJECTIVE: To present a case series to elucidate a novel technique that involves the creation of an arcuate retinotomy in the treatment of large macular holes after failed primary repair. PATIENTS AND METHODS: retrospective chart review. Six eyes (six patients) with large macular holes, all of which had failed primary repair, underwent 25 gauge pars plana vitrectomy revision coupled with full thickness arcuate retinotomy temporal to the macular hole and fluid-gas exchange. The main outcome measure was anatomic macular hole closure based on optical coherence tomography (OCT), with visual acuity and visual field evaluation as secondary outcome measures. RESULTS: Five of the six patients (83%) had successful hole closure with three of the six patients (50%) exhibiting improvement in visual acuity. CONCLUSION: Arcuate retinotomy is a new approach that may aide in the repair of large macular holes not otherwise amenable to closure with traditional techniques.

25-gauge pars plana vitrectomy with medium-term postoperative perfluoro-n-octane tamponade for inferior retinal detachment.

BACKGROUND AND OBJECTIVE: To describe a series of patients with inferior retinal detachments managed with primary 25-gauge pars plana vitrectomy (PPV), 2 to 3 weeks of postoperative perfluoro-n-octane (MT-PFO) tamponade, and upright positioning followed by secondary PPV and PFO removal. PATIENTS AND METHODS: Interventional case series of 157 patients with inferior retinal detachments with and without proliferative vitreoretinopathy. Eyes were treated with 25-gauge PPV, endophotocoagulation, and MT-PFO tamponade. Patients underwent postoperative upright positioning followed by repeat PPV and PFO removal in a planned, staged procedure. RESULTS: Mean follow-up was 32 ± 4.6 months. Successful reattachment was achieved in 87.5% of 159 eyes. Main initial postoperative complications were persistent intraocular pressure (IOP) elevation in 34% (n = 54), excessive inflammation in 27% (n = 43), and PFO in the anterior chamber in 21% (n = 34). Additional complications occurring after PFO removal included cataract surgery in 16% (n = 22) of initially phakic eyes, redetachment in 13% (n = 21), and need for filtering surgery in 6% (n = 10). Logistic regression analysis revealed macula status (P = .003) and progression to filtering surgery (P = .001) as significant factors predicting visual outcome at 1-year follow-up. CONCLUSION: MT-PFO tamponade and upright head positioning may be efficacious for inferior retinal detachment repair in patients unable to assume face-down postoperative positioning. Anatomic and visual outcomes are similar to previously described reattachment procedures. A characteristic granulomatous inflammatory reaction presents in some patients, but does not appear to leave long-term visual or anatomic sequelae. Persistent IOP elevation and progression to filtering surgery may occur in a small percentage of patients and portends a worse visual outcome.

Delayed onset inner nuclear layer cystic changes following internal limiting membrane removal for epimacular membrane.

PURPOSE: To report the rare occurrence of new inner nuclear layer cystic spaces occurring in eyes treated with pars plana vitrectomy (PPV) and internal limiting membrane (ILM) removal for idiopathic epimacular membrane (EMM). MATERIALS AND METHODS: Consecutive patients with EMM without preoperative retinal cystic changes undergoing PPV with ILM peeling were retrospectively evaluated. Patients developing a characteristic inner nuclear layer cystic change were analyzed. RESULTS: Inner nuclear layer cystic changes appeared in eight of 768 (1.04 %) eyes at a mean postoperative time period of 3.2 ± 0.89 months. No leakage or pooling was demonstrated on postoperative fluorescein angiography. Morphologic characteristics included vertically elongated hyporeflectant spaces within the inner nuclear layer on spectral domain optical coherence tomography (SD-OCT). CONCLUSIONS: A minority of patients undergoing PPV with ILM peeling develop new, delayed onset, postoperative inner nuclear layer cystic spaces with a characteristic SD-OCT appearance and no evidence of angiographic leakage.

Pars plana vitrectomy with medium-term postoperative perfluoro-N-octane for recurrent inferior retinal detachment complicated by advanced proliferative vitreoretinopathy.

PURPOSE: To describe a series of patients with recurrent inferior retinal detachment complicated by proliferative vitreoretinopathy (PVR) treated with pars plana vitrectomy and postoperative perfluoro-n-octane (PFO). METHODS: Consecutive patients with recurrent inferior retinal detachment and Grade C PVR were prospectively treated with 25-gauge pars plana vitrectomy and postoperative "medium-term" PFO for 2- to 3-week duration. All patients had subsequent PFO removal in a planned staged procedure. RESULTS: Forty-four eyes of 44 patients were included. Mean follow-up time was 30.71 ± 12.92 months. Successful reattachment was achieved in 86% of eyes (38/44). Reattachment rates were statistically equivalent between eyes with and without previous scleral buckle. Reasons for redetachment were recurrent inferior PVR (four of six) and new superior break without PVR (two of six). Additional complications observed were cataract progression requiring surgery (42%), persistent intraocular pressure elevation (36%), and transient inflammation (32%). Macula-off status (P = 0.02) and persistent intraocular pressure elevation (P = 0.02) were factors associated with worse visual outcome. CONCLUSION: Medium-term PFO was found to be an efficacious technique for operative management of recurrent inferior retinal detachments complicated by Grade C PVR. The method of primary repair (scleral buckle vs. pars plana vitrectomy) did not affect reattachment rates. Transient inflammation and intraocular pressure elevation are potential complications associated with this technique. Persistent intraocular pressure elevation was associated with worse visual outcome.

Comparison of observation, intravitreal bevacizumab, or pars plana vitrectomy for non-proliferative type 2 idiopathic macular telangiectasia.

PURPOSE: To compare visual and anatomic outcomes in eyes with type 2 idiopathic macular telangiectasia (Mactel) treated with either intravitreal bevacizumab (IVB), observation, or pars plana vitrectomy (PPV) with internal limiting membrane removal. METHODS: Retrospective, consecutive, interventional case series of phakic patients with Mactel. Best-corrected Snellen visual acuity (BCVA) and complete ophthalmic exam was obtained prior to treatment and at subsequent 3-month intervals for a minimum of 6 months. Fluorescein angiographic and spectral-domain optical coherence tomography features were examined, and compared to BCVA at treatment initiation and follow-up. RESULTS: Fifty-six eyes of 28 patients were evaluated. Mean age was 65 ± 12 years, and mean follow-up was 24 ± 13 months. Patients were treated with either observation (n = 33), IVB (n = 15), or PPV (n = 8). Mean number of treatments for the IVB group was 2.5 ± 3.5 intravitreal injections. No significant differences in BCVA change were observed between treatment groups via one-way ANOVA (p = 0.49). Presence of inner retinal cysts was not correlated to BCVA (p > 0.05). Discontinuous outer nuclear layer was significantly related to worse initial and final vision, but not to BCVA change. CONCLUSION: IVB and PPV with ILM removal appear ineffective in improving visual outcome in eyes with non-proliferative Mactel. SD-OCT evidence of disrupted foveal outer nuclear layer is related to decreased BCVA, but not related to BCVA change following treatment.

Pars plana vitrectomy with internal limiting membrane removal in type 2 idiopathic macular telangiectasia.

PURPOSE: To report the anatomical and visual outcomes in a series of patients with type 2 idiopathic macular telangiectasia (mactel) and cystic foveal changes treated with pars plana vitrectomy and internal limiting membrane removal. METHODS: Five eyes of 3 patients with mactel were treated with 25-gauge sutureless transconjunctival pars plana vitrectomy and internal limiting membrane peeling. Complete clinical examination, including serial spectral-domain optical coherence tomography, was performed preoperatively and postoperatively. RESULTS: Two of 5 eyes had stable vision at 2-year follow-up with continued evidence of foveal cystic spaces on spectral-domain optical coherence tomography. Two eyes experienced significant visual improvement despite continued evidence of foveal anatomical disruption. One eye experienced slight visual decline and full-thickness macular hole formation. CONCLUSION: Surgical intervention involving pars plana vitrectomy with internal limiting membrane peeling may be a treatment option for select cases of mactel. Pars plana vitrectomy does not seem to alter the underlying disease process in mactel but may stabilize visual decline or lead to improved vision in some patients.

Spectral domain optical coherence tomography characteristics of retained subretinal perfluoro-n-octane.

PURPOSE: To describe the spectral domain optical coherence tomography characteristics of retained subretinal perfluoro-n-octane (SR-PFO) after vitreoretinal surgery. METHODS: Consecutive patients referred for retained SR-PFO after pars plana vitrectomy for retinal detachment were prospectively evaluated with spectral domain optical coherence tomography. The clinical findings, fundus autofluorescence, spectral domain optical coherence tomography images, and anatomical outcomes, were reviewed. RESULTS: Three consecutive patients presented to the vitreoretinal service with history of retinal detachment and pervious vitrectomy with intraoperative PFO. Subretinal PFO bubbles were observed on clinical examination and imaging studies, including spectral domain optical coherence tomography. Case 1 had subfoveal PFO removal and developed a postoperative choroidal neovascular membrane. Case 2 had multiple foci of SR-PFO after giant retinal tear repair. Case 3 had superotemporal SR-PFO that remained stable for several years without migration or other associated pathology. CONCLUSION: Subretinal PFO appears as a low-reflectance spherical mass, often with a pinpoint focus of hyperreflectance at the apex of the bubble. Optical coherence tomography details beneath subretinal PFO appear relatively hyperreflectant compared to adjacent tissue. Long-term observation revealed preferential absence of the outer retinal layers with no nerve fiber layer erosion, subretinal inflammatory precipitates, or proliferative vitreoretinopathy. Some evidence of retinal pigment epithelial disruption was observed but seemed to remain stable or improve with observation. The authors suggest management involving follow-up with close observation in SR-PFO unless present in the subfoveal region. Choroidal neovascular membrane can occur after SR-PFO removal and may be successfully managed with intravitreal bevacizumab.

Traction retinal detachment under silicone oil treated with two-port, 25-gauge pars plana vitrectomy and scissor segmentation in a child with familial exudative vitreoretinopathy.

PURPOSE: To describe a method of 25-gauge pars plana vitrectomy (PPV) with posterior hyaloid scissor segmentation in a child with recurrent retinal detachment caused by vitreous traction under silicone oil. METHODS: A two-port, 25-gauge PPV was performed under previously instilled silicone oil with retinotomy and internal drainage of subretinal fluid. RESULTS: A 5-year-old monocular girl with familial exudative vitreoretinopathy presented with decreased vision and a recurrent retinal detachment 3 weeks after PPV and air-silicone exchange for rhegmatogenous retinal detachment. Ophthalmoscopy, fundus photography, and optical coherence tomography revealed persistent posterior hyaloid adherent to multiple retinal foci inducing a tractional detachment. The patient underwent two-port PPV revision with scissor segmentation of vitreous traction. Follow-up examination at 3 months revealed the absence of vitreous traction, an attached retina, and markedly improved vision. CONCLUSION: Posterior vitreous sequestered under silicone oil inducing consecutive retinal detachment may be successfully managed with two-port interface PPV and scissor segmentation of vitreous traction.

Intravitreal fluorinated gas preference and occurrence of rare ischemic postoperative complications after pars plana vitrectomy: a survey of the american society of retina specialists.

Objective. To perform a survey of the American Society of Retina Specialists (ASRS) regarding the use of vitreous cavity fluorinated gas as an adjunct to pars plana vitrectomy for retinal detachment or macular hole repair. Methods. A multiple-choice online questionnaire was administered to members of ASRS. Physician experience, gas preference for vitrectomy, and categorical estimate of observation of blinding postoperative ischemic events were recorded. Results. 282 questionnaires were completed. Mean years in vitreoretinal practice were 15 ± 10. A decrease in yearly vitrectomy volume was associated with increased number of years in practice (P = 0.011). Greater than 95% of respondents preferred fluorinated gas to air alone for both retinal detachment and macular hole repair. 38% of respondents reported at least one observation of a blinding ischemic postoperative event. Overall estimated incidence of blinding postoperative ischemic event was 0.06 events/year in practice. Conclusions. Currently, C3F8 and SF6 are the postoperative gas preference for ASRS respondents, in contrast to previous North American surveys. The occurrence of blinding ischemic events appears unrelated to number of years in practice, was reported by less than half of those surveyed, and has occurred at an infrequent rate of approximately once for every ten years of practice for those observing the phenomena.

Orbital manifestations of Noonan syndrome.

Noonan syndrome describes a rare multisystem condition that manifests with Turner syndrome phenotype combined with numerous systemic and facial characteristics. The most common systemic findings include cardiac defects, short stature, chest deformity, hearing loss, and bleeding diatheses. Patients with Noonan syndrome are also at a greater risk of developing various types of malignancies. Facial characteristics of Noonan syndrome consist of broad forehead, low-set ears, short and webbed neck, and low hairline. External ocular findings include hypertelorism, ptosis, epicanthal folds, antimongoloid slant, downward-sloping palpebral fissures, and malar flattening. In this case series and review of the literature, the authors present 2 cases of Noonan syndrome that embody the diverse spectrum of orbital manifestations associated with this rare disorder. The first case demonstrates more profound orbital pathology, including bilateral orbital giant cell reparative granulomas, proptosis, hypertelorism, shallow orbits, upper eyelid ptosis, and lower eyelid retraction. The second case displays classic Turner-syndrome-like facial characteristics including a broad forehead with low hairline, low-set ears, and short and webbed neck. Orbital findings were subtle and consisted of bilateral lower eyelid retraction and shallow orbits. In conclusion, Noonan syndrome is a rare congenital disorder with a wide spectrum of clinical findings. Various intraorbital and extraorbital tumors may occur in patients with Noonan syndrome, with giant cell reparative granuloma being the most commonly encountered. In patients with orbital tumors and eyelid retraction, the authors describe successful treatment through decompression, tumor extraction, and lower eyelid retractor release. Patients who present with ocular irritation and exposure due to less severe lower eyelid malposition may be successfully treated with lower eyelid retraction repair combined with lateral internal tarsoconjunctival tarsorrhaphy.