Surgical Management of Full-Thickness Macular Holes in Macular Telangiectasia Type 2: A Global Multicenter Study.

PURPOSE: To report on macular hole repair in macular telangiectasia type 2 (MacTel2). DESIGN: Global, multicenter, retrospective case series. PARTICIPANTS: Patients undergoing surgery for MacTel2-associated full-thickness macular hole (MTMH). METHODS: Standardized data collection sheet distributed to all surgeons. MAIN OUTCOME MEASURES: Anatomic closure and visual outcomes of MTMH. RESULTS: Sixty-three surgeries in 47 patients with MTMH were included from 30 surgeons. Mean age was 68.1 years, with 62% female, 72% White, 21% East or South Asian, 2% African American, and 2% Hispanic or Latino. Procedures included 34 internal limiting membrane (ILM) peeling alone, 22 ILM flaps, 5 autologous retinal transplantations (ARTs), 1 retinotomy, and 1 subretinal bleb. For ILM peeling, preoperative visual acuity (VA) was 0.667 ± 0.423 logarithm of the minimum angle of resolution (logMAR). Minimum hole diameter (MHD) was 305.5 ± 159.4 µm (range, 34-573 µm). Sixteen of 34 ILM peels (47%) resulted in MTMH closure. At postoperative month 6, VA was stable at 0.602 ± 0.516 logMAR (P = 0.65). VA improved by at least 2 lines in 43% and at least 4 lines in 24%. For ILM flaps, preoperative VA was 0.878 ± 0.552 logMAR. MHD was 440.8 ± 175.5 µm (range, 97-697 µm), which was significantly larger than for ILM peels (P < 0.01). Twenty of 22 ILM flaps (90%) resulted in MTMH closure, which was significantly higher than for ILM peels (P < 0.01). At postoperative month 6, VA improved to 0.555 ± 0.405 logMAR (P < 0.05). VA improved by at least 2 lines in 56% and at least 4 lines in 28%. For ARTs, preoperative VA was 1.460 ± 0.391 logMAR. MHD was 390.2 ± 203.7 µm (range, 132-687 µm). All 5 ARTs (100%) resulted in MTMH closure. At postoperative month 6, VA was stable at 1.000 ± 0.246 logMAR (P = 0.08). Visual acuity improved at least 2 lines in 25%. CONCLUSIONS: Surgical closure of macular holes improved VA in 57% of MTMHs. Internal limiting membrane flaps achieved better anatomic and functional outcomes than ILM peeling alone. Autologous retinal transplantation may be an option for refractory MTMHs. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Lifting the White Walker's curse-Management of Coats' disease.

Background: Coats' disease, described by George Coats in the early 1900s, is an idiopathic unilateral retinal vascular abnormality with exudation occurring in young males. It is characterized by retinal telangiectasia with intraretinal or subretinal exudation. Coats' disease is mostly diagnosed in the first to the second decade of life, with a common presentation of leukocoria. Younger patients have a more severe presentation and are associated with poor visual prognosis. Management of Coats' disease varies from observation, cryotherapy with anti-VEGFs (Vascular Endothelial Growth Factor), and surgery to enucleation. The mode of treatment depends on the age of presentation, the severity of the disease, and the stage of the disease. Through this video, we describe the clinical features, pathology, and surgical management of a 2-year-old child with grade 3B of Coats' disease. Purpose: To demonstrate successful surgical management of grade 3B of Coats' disease in a 2-year-old boy. Synopsis: Coats' disease mostly presents with a diagnostic dilemma due to its varied presentation. Early detection and treatment are the keys to salvaging the eye as well as the vision, hence, avoiding dreadful complications such as neovascular glaucoma or phthisis bulbi. We demonstrate successful surgical management of a child who presented with grade 3B of Coats' disease. Highlights: Through this video, we aim to describe the clinical features, pathology, and surgical management of a 2-year-old child with grade 3B of Coats' disease. Combination of external drainage with vitrectomy, challenges faced, and the importance of visual rehabilitation postoperatively. Video Link: https://youtu.be/0obpVTOkKKs.

Clinicopathological Correlations in Enucleated Globes of Late-Stage Coats Disease with a Review of the Literature.

BACKGROUND: Coats disease may cause diagnostic dilemma because of its variable clinical presentation that can be suspicious of retinoblastoma. Late sequelae of the disease are blinding with possible enucleation. We demonstrate the main histopathological findings of Coats enucleated eyes with literature review. METHODS: This was a retrospective study of all enucleated globes diagnosed as Coats disease over 30 years and were reviewed by two pathologists. The corresponding demographic data, clinical presentation, pre-operative clinical impression, and indication for enucleation were collected. Descriptive analysis of our own series data was performed. Our findings were then correlated to published data that were collected from 1983 to 2021 from the PUBMED database in English-written language. Shields classification was used as an inclusion criterion for the published reports to be analyzed. RESULTS: We had seven enucleated globes with Coats disease. Mean age at presentation was 3.2 years (range 3 months to 9 years). Male predominance was observed in 6 and all cases were unilateral. Strabismus was the most common initial presentation (57%, n = 4), followed by leukocoria (43%, n = 3). Indication for enucleation was mostly suspected retinoblastoma (57%, n = 4). Four eyes were classified as stage 4, and 2 had advanced stage 5 changes. Histopathologically, subretinal fluid with lipid-laden macrophages was seen in all cases, the anterior chamber was shallow in 5/7 with angle neovascularization in 2/7. Telangiectatic vessels were clearly observed in 4/7. CONCLUSION: Coats disease is a potentially visually disabling disease that is mostly unilateral in 95%, has male predominance of 81%, and wide age range with a mean of 17 years. In Saudi Arabia, the disease seems to present at younger age, tends to be more advanced, and may be indistinguishable from retinoblastoma at the time of diagnosis. Shields staging of Coats is highly recommended to be followed clinically to unify the pathways for treatment and to correlate the concluded outcomes.

Subthreshold micropulse laser for adult onset Coats' associated exudative maculopathy.

PURPOSE: To report subthreshold micropulse laser as novel treatment modality for exudative maculopathy associated with adult Coats' disease. METHODS: A 27-year old, young male presented with exudative maculopathy in adult onset Coat's disease with profound diminution of vision. Spectral domain-optical coherence tomogram (SD-OCT) revealed macular oedema with significant subfoveal hard exudates with massive subretinal exudations temporal to macula. He was planned for anti-VEGF injection to treat macular oedema in order to preserve vision but developed conjunctivitis which made any interventional procedure impossible. Patient was managed with subthreshold micro pulse laser using 532 nm green laser. RESULTS: Patient had significant improvement in vision, resolution of macular oedema as well as hard exudate without any adverse effects on fovea. CONCLUSION: Novel treatment approach in form of subthreshold micro pulse laser could be an alternative treatment modality for exudative maculopathy associated with adult onset Coat's disease, especially in cases where other available interventional treatment options are not possible.

Rhegmatogenous retinal detachment in Coats' disease: a case report.

BACKGROUND: Coats' disease was originally defined as a unilateral idiopathic exudative retinopathy in young males, characterized by abnormal retinal vascular telangiectasia with intraretinal and subretinal lipid exudation. The retinal detachment is usually exudative. Herein, we describe a case of rhegmatogenous retinal detachment with detectable retinal break in a patient with Coats' disease. CASE PRESENTATION: A 15-year-old Indo-Aryan male patient presented with sudden painless diminution of vision in his right eye of 4 days duration. Upon examination, the anterior segment in both eyes and left fundus was within normal limits. Dilated fundus evaluation of the right eye revealed telangiectasia of the retinal vessels, with subretinal exudation in superotemporal and superonasal quadrants and presence of subretinal fluid in the superotemporal area extending into fovea. There was also presence of single flap horseshoe tear in the superotemporal quadrant at around the 10 o'clock position in the equatorial region, with no secondary changes. The retina was reattached with encircling band buckle combined with vitrectomy and silicon oil tamponade. Seven months post vitrectomy, lenticular opacification developed, for which he underwent silicon oil removal, along with lens aspiration and implantation of foldable intraocular lens. Over the period of 1 year, his best corrected visual acuity improved from 6/60 to 6/18 in the affected eye at the last follow-up visit. The recovery was uneventful following the subsequent surgery. CONCLUSION: Coats' disease has a remarkable diversity in clinical presentation and morphology. The disease can also present with an underlying break, which may not be attributed to any iatrogenic modality. The treatment modalities in coats' disease should be tailored individually due to the low incidence of the disease and the great variation in severity upon presentation. Prompt management restores the best possible anatomical outcome and maintains good vision.

Vitrectomy and external drainage of subretinal fluid containing high concentration of vascular endothelial growth factor for advanced coats disease.

This study investigated the surgical outcomes of Coats disease and the role of external drainage (XD) of subretinal fluid (SRF). The study is a multicenter retrospective interventional case series of 26 consecutive eyes of 26 patients who underwent surgeries for advanced Coats disease with retinal detachment. Main outcomes measured were: 1) comparison of complete SRF resolution with or without XD, and 2) variables that were associated with functional postoperative best-corrected visual acuity (BCVA) defined as BCVA of 0.1 or better, 3) intraocular vascular endothelial growth factor (VEGF) levels. Complete SRF resolution was achieved in all 14 eyes in which XD had been performed and in 75% of 12 eyes in which XD had not been performed (P = .03). Multivariable logistic regression analysis revealed that initial BCVA was the only variable associated with functional postoperative BCVA (odds ratio 3.24, 95% CI 0.93-11.33; P = .04). Markedly elevated VEGF levels were noted in the SRF compared with those in the vitreous humor (49,760 ± 52,990 vs. 707 ± 611 pg/mL, P = .03). XD seems to provide better anatomical success than without XD in the treatment of advanced Coats disease as XD could effectively eliminate substantial amount of VEGF in the SRF.

Internal limiting membrane graft in full-thickness macular hole secondary to macular telangiectasia type 2.

We present a case report of an autologous internal limiting membrane (ILM) graft transposition technique in a 60-years-old female patient who with a full-thickness macular hole (FTMH) secondary to idiopathic macular telangiectasia type 2 (MacTel type 2). To our knowledge, our case is the first report of ILM graft transposition to treat persistent FTMH secondary to MacTel type 2. MacTel type 2 is an acquired disease characterized by macular capillary abnormalities and retinal neurodegeneration. FTMH is an infrequent complication of MacTel type 2. Pars plana vitrectomy (PPV) with ILM peeling and gas endotamponade is the standard treatment of FTMH to help restore macular anatomy and visual function. The technique of autologous ILM graft transposition is a useful alternative to repair large, persistent macular holes that have failed to close with previous ILM peeling. In the present case, our patient with persistent FTMH secondary to MacTel type 2 after the previous standard macular hole surgery was admitted to our clinic. We performed 23-gauge PPV with extramacular ILM peeling and ILM graft transposition into the macular hole. 2 months after the surgery, her visual acuity had improved and anatomic closure of the FTMH was observed. In our opinion, ILM grafting might support reorganization of the retinal structure that has been affected by Muller cell degeneration.

FUNCTIONAL AND STRUCTURAL EFFECTS OF NONDAMAGING RETINAL LASER THERAPY FOR MACULAR TELANGIECTASIA TYPE 2: A Randomized Sham-Controlled Clinical Trial.

PURPOSE: Macular telangiectasia (MacTel) Type 2 is a progressing neurovascular disease of the macula, currently lacking effective treatment. This study assessed the effect of nondamaging retinal laser therapy (NRT) compared with sham. METHODS: Twelve MacTel patients were enrolled in this double-masked, controlled, randomized clinical trial. For the nine patients with both eyes eligible, one eye was randomized to NRT or sham and the other received alternate treatment. For three patients with only one eye eligible, that eye was randomly assigned either NRT or sham. Ellipsoid zone disruption, best-corrected visual acuity, and macular automated perimetry at 12 months served as structural and functional measures. RESULTS: Eleven eyes were randomized to sham and 10 to NRT. Baseline best-corrected visual acuity was 66 letters (20/50) for sham and 72 letters (20/40) for NRT (P = 0.245). Ellipsoid zone disruption area was 298 µm2 in sham and 368 µm2 in NRT (P = 0.391). At 12 months, ellipsoid zone disruption increased by 24% in sham and decreased by 34% in NRT (P < 0.001). Best-corrected visual acuity measures remained stable during follow-up compared with baseline. At 1 year, the mean macular sensitivity was 28 dB in the NRT group, compared with 26 dB in sham. CONCLUSION: Nondamaging retinal laser therapy was safe and well tolerated in patients with MacTel and resulted in structural and functional improvements, which could represent a protective effect of laser-induced hyperthermia. Longer follow-up and larger number of patients should help corroborate these effects.

External Drainage Alone Versus External Drainage With Vitrectomy in Advanced Coats Disease.

PURPOSE: To evaluate the efficacy of transscleral drainage of subretinal fluid (TDSRF) with and without pars plana vitrectomy (PPV), which are performed as an adjunct to ablative therapies in advanced Coats disease. DESIGN: Retrospective, comparative case series. PATIENT POPULATION: A total of 31 eyes from 31 consecutive patients with advanced Coats disease at an average age of 47.8 ± 34.2 (2-156) months at the time of the surgery, who underwent TDSRF alone or in combination with PPV. METHODS: Main outcome measurements were LogMAR visual acuity values, anterior and posterior segment findings, need for further surgery, laser photocoagulation, and anti-vascular endothelial growth factor (anti-VEGF) treatment. Anatomical success was defined as the maintenance of retinal reattachment without any further surgery during follow-up. RESULTS: Sixteen patients underwent TDSRF alone, and 15 patients underwent combined TDSRF and PPV. Mean follow-up time was 34.8 ± 32.9 months (6-128). Anatomical success rate of combined TDSRF and PPV was higher than that of TDSRF alone (93.8% vs 75%, respectively). The incidence of epiretinal membrane formation, number of laser photocoagulation procedures, and anti-VEGF treatments were statistically significantly higher in the group that underwent TDSRF alone than in those who had combined TDSRF and PPV in the long term. There was an improvement in vision in only 4 eyes (12.9%) (all >3 years old at presentation) during the follow-up. CONCLUSIONS: Combined TDSRF and PPV appears to be more effective in controlling the disease than TDSRF alone, as an adjunct to ablative procedures for the treatment of advanced Coats disease. The need for additional treatment is much less with the addition of PPV.

Neglected Coats Disease.

Coats disease is a rare, non-hereditary retinal vascular abnormality that typically presents in the first two decades of life and is characterized by idiopathic retinal telangiectasia with progressive exudation. The authors describe a patient with Coats disease in which the family neglected treatment, demonstrating the natural course of this disease. [J Pediatr Ophthalmol Strabismus. 2020;57:e82-e85.].

Coats retinopathy with pachychoroid and central serous chorioretinopathy in the fellow eye.

A 37-year-old man presented with blurring of vision and scotoma in the left eye since last 2 weeks and has a history of poor vision in his right eye since childhood. On examination, his best corrected visual acuity in the right eye was counting finger 1 m and 20/20, N/12 in the left eye. Fundus examination revealed features of Coats disease in the right eye and central serous chorioretinopathy (CSCR) in the left eye. He was given intravitreal triamcinolone acetonide injection in the right eye for reducing subretinal exudation. Simultaneously, focal laser photocoagulation was done to the CSCR leaks in the left eye to prevent the possible worsening of CSCR due to steroid injection in the fellow eye. He underwent cryotherapy to the retinal telangiectatic vessels 3 weeks after steroid injection in the right eye. Optical coherence tomography (OCT) of both eyes showed pachy vessels with a compression of overlying choriocapillaries. After 1 month, the condition of the right eye was unchanged and the CSCR in the left eye was completely resolved with the recovery of near visual acuity.

Efficacy of Macular Hole Surgery in Patients with Idiopathic Macular Telangiectasia Type 2.

PURPOSE: To compare visual acuity (VA) and OCT outcomes in patients with idiopathic macular telangiectasia (IMT) type 2 who underwent pars plana vitrectomy (PPV) surgery for full-thickness macular holes (FTMHs) versus those who elected to be medically managed (MM) without surgery. DESIGN: Comparative retrospective case series. PARTICIPANTS: Patients with IMT type 2 and FTMH. METHODS: We reviewed records within an 11-year period and collected data on VA, OCT changes, development of choroidal neovascularization, and length of follow-up. The VA measurements were standardized from Snellen to logarithm of the minimum angle of resolution units for statistical analysis. Two-sample t tests were used to analyze VA data. OCT changes were assessed by a single masked retinal specialist. RESULTS: There were 12 eyes in the PPV group and 26 eyes in the MM group. There was no statistically significant VA improvement in either group between initial VA recording and last follow-up. The PPV group had no significant change in VA between the preoperative visit and the visits at 3 or 12 months. OCT scans improved by 1 step in 10 patients in the PPV group. None of the patients in the MM group had OCT improvement. Choroidal neovascularization developed in 1 eye in the PPV group and 5 eyes in the MM group. CONCLUSIONS: There was no significant change in VA in patients who opted to have PPV to treat their IMT type 2 and FTMH compared with those who did not undergo surgery. OCT scans improved by qualitative judgment in patients who underwent surgery compared with those who opted for medical management.

A 10-year review of histopathological findings of enucleated eyes from a tertiary-care center, Pakistan.

OBJECTIVE: To determine the histopathology of enucleated eye specimens. METHODS: The 10-year inventory was conducted at Aga Khan University Hospital, Karachi, and comprised all histopathology reports of enucleated eye specimens received from January 2007 to December 2016 by the Section of Histopathology, Department of Pathology. SPSS 19 was used for data analysis. RESULTS: A total of 232 ocular tissue specimens from 231 patients were evaluated. Ocular tumours were the most common histopathological diagnosis 186(80%). Among tumours, retinoblastoma was the most common 137(59%) followed by malignant melanoma 31(13%) and squamous cell carcinoma 15(6.5%). Overall, 16(6.9%) specimens had degenerative changes secondary to different ocular disorders. Staphyloma and Coat's disease was diagnosed in 3(1.3%) cases each. In 4(1.7%) cases, there was no formal diagnosis. CONCLUSIONS: Early identification of tumours may allow for conservative management and limit the need for enucleations.

Visual outcomes after treatment in pediatric patients with Coats' disease.

OBJECTIVE: To report visual outcomes for children with Coats' disease after treatment. DESIGN: Retrospective case series. PARTICIPANTS: Pediatric patients with Coats' disease treated between 2000 and 2018 at a tertiary care pediatric hospital. METHODS: Review of medical records. The primary outcome was visual acuity at final follow-up. Anatomical outcomes, retreatment, and risk factors for a poor outcome were also assessed. RESULTS: There were 30 patients with Coats' disease. All cases were unilateral, and 28 (93%) were male. At presentation, 14 (47%) had stage 2 disease (retinal exudates) and 16 (53%) had stage 3 disease (subtotal or total exudative retinal detachment). All patients underwent laser photocoagulation and (or) cryopexy as primary treatment, combined with antivascular endothelial growth factor injection in 7 patients, posterior sclerotomy in 5 patients, and pars plana vitrectomy in 1 patient. Retreatment was required in 16 (53%) patients. After a median follow-up of 3.8 years, visual acuity was 20/50 or better in 6 patients (20%), 20/60 to 20/150 in 3 (10%), 20/200 to counting fingers in 8 (23%), and hand motion or worse in 14 (47%). Greater severity of disease at presentation was significantly associated with a poor visual outcome (p = 0.0001). In terms of complications, 7 (23%) eyes developed cataracts and 2 (7%) progressed to phthisis bulbi, but no patients required enucleation. CONCLUSIONS: The visual prognosis for children with Coats' disease remains poor, particularly in patients with more severe disease at presentation. The risk of severe complications and enucleation is low after treatment.

Predictors of enucleation in Coats disease: analysis of 259 eyes of 259 patients at a single center.

PURPOSE: To evaluate the effects of clinical features associated with enucleation in eyes with Coats disease. METHODS: The medical records of all patients with Coats disease at the Ocular Oncology, Wills Eye Hospital from November 1, 1973, to July 31, 2018, were reviewed retrospectively. The clinical features pertaining to need for ultimate enucleation and time to enucleation were compared. RESULTS: The records of 351 eyes were reviewed, of which 259 had follow-up at our center and 32 (12%) were managed with enucleation. Reasons for enucleation included neovascular glaucoma (n = 24 [75%]), possible tumor (6 [19%]), and phthisis bulbi (2 [6%]). Compared to nonenucleated eyes, enucleated eyes had more extensive clock hour involvement of telangiectasia (P < 0.001), light bulb aneurysms (P < 0.001), exudation (P < 0.001), and subretinal fluid (P < 0.001). On adjusted analysis by binomial logistic regression, variables predictive of enucleation included presence of iris neovascularization (P = 0.01), ultrasonographic retinal detachment (P = 0.004), open-funnel retinal detachment (P = 0.04), closed-funnel retinal detachment (P = 0.01), ultrasonographic elevation of subretinal fluid by millimeters (P = 0.001), and angiographic extent of light bulb aneurysms by clock hours (P = 0.02). By Kaplan-Meier analysis of 4-year cumulative risk of enucleation, risk factors for enucleation included presence of iris neovascularization (hazard ratio [HR] 31.0; P < 0.001), ultrasonographic retinal detachment (HR 56.2; P < 0.001), open-funnel retinal detachment (HR 2.7; P = 0.01), and closed-funnel retinal detachment (HR 4.5; P < 0.001). CONCLUSIONS: Clinical features that predict risk of and time to enucleation in eyes with Coats disease include iris neovascularization, ultrasonographic presence and millimeter-elevation of retinal detachment, and angiographic extent of light bulb aneurysms.

Adult Coats' Disease, Dubin-Johnson Syndrome, and the Search for Targeted Therapies.

Coats' disease is nonhereditary retinal vascular disorder characterized by telangiectatic retinal vessels with prominent aneurysmal changes and exudation. A conclusive etiology has not yet been determined. In this retrospective case report and literature review, a 64-year-old male with Dubin-Johnson syndrome presented with unilateral retinal vascular changes and exudation consistent with a diagnosis of adult Coats' disease. The authors conclude that patients with Dubin-Johnson syndrome carry mutations in a multidrug resistance associated protein (MRP). MRPs are also expressed in the retina, retinal pigment epithelium, and vascular endothelium, where they export toxins and metabolites, and may serve as a therapeutic target. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:318-321.].

Diagnosis and treatment of bilateral Coats disease in a 5-year-old girl.

A 5-year-old girl presented with decreased vision and outward deviation of her right eye. Fundus examination revealed multiple hard exudates in the macula in the right eye and nasal to the disk in the left eye. The patient was lost to follow-up in the near term but presented 9 months later with reduced vision and an increase in exudates in both eyes. RetCam fluorescein angiography confirmed the diagnosis of bilateral Coats disease.

RETINAL TELANGIECTASIA IN PATIENTS WITH PATHOLOGIC MYOPIA: A CASE SERIES.

PURPOSE: To describe the characteristics of retinal telangiectasia in eyes with pathologic myopia. METHODS: The study included 10 patients (18 eyes) who were diagnosed with pathologic myopia combined with retinal telangiectasia. The patients visited our retinal clinic every 3 months. Nine eyes underwent vitrectomy for vision-threatening complications after diagnosis. All eyes underwent comprehensive ophthalmologic examinations including multimodal retinal imaging at presentation and at each follow-up. RESULTS: Retinal telangiectasia in pathologic myopia was characterized by saccular aneurysmal dilatation of the capillary bed without hard exudates in color fundus photographs and hyporeflective saccular structure in infrared reflectance fundus photographs, and it was filled in the early retinal arteriovenous phase with minimal dye leakage in the late phase of fundus fluorescein angiography. Spectral domain optical coherence tomography and optical coherence tomographic angiography showed that retinal telangiectasia was primarily located in the superficial retina, together with myopic traction maculopathy. In the 9 eyes that underwent vitrectomy, the retinal telangiectasia regressed within 3 months of surgery. Retinal telangiectasia remained stable in the other nine eyes, but these eyes were at risk of spontaneous bleeding. CONCLUSION: Retinal telangiectasia is a relatively quiescent and uncommon disorder in patients with pathologic myopia that might be closely related to myopic traction maculopathy.