Long-term anatomical and functional outcomes of surgical treatment of retinal complications in children and adolescents with Stickler syndrome between 2004 and 2021.

BACKGROUND: Stickler syndrome (STL) is an inherited progressive connective tissue collagen disorder. STL is the most common hereditary cause of retinal complications, retinal tears, and the development of retinal detachment (RD) in childhood. The aim of the study was to evaluate the long-term anatomical and functional results of surgical treatment of retinal complications in children and adolescents affected by STL. METHODS: A retrospective, single-center study was performed a cohort of children with STL who underwent retinal surgery between 2004 and 2021. RESULTS: The study group consisted of nine children; the mean age at the time of the retinal tear with/without retinal detachment was 7.2 (2-10) years, and the mean follow-up period was 9.6 (5-16) years. Pathogenic variants COL2A1 (5 children) and COL11A1 (3 children) were confirmed in our cohort. In total, we operated on 13 eyes, 11 eyes with complicated RD and two eyes with multiple retinal defects, but without RD. At the end of the follow-up period, an attached retina was achieved 77% (10 eyes) with or without silicone oil tamponade: cryopexy alone was successful in one eye (10%), scleral buckling (EB) in five eyes (50%), and vitrectomy with silicone oil tamponade combined with EB in four eyes (40%). The mean number of surgeries was 2.3 per eye. The resulting best corrected visual acuity ranged from 0.03 to 0.1 in one eye, from 0.16 to 0.4 in two eyes, and from 0.5 to 1.0 in 7 eyes. CONCLUSION: Repair of retinal tears with/without retinal detachment in patients with Stickler syndrome often requires multiple surgeries with combinations of cryopexy, scleral buckling, and/or vitrectomy with silicone oil tamponade. Treatment of the ocular complications arising from STL requires long-term comprehensive care.

Surgical management of complicated retinal detachment in a case of retinal hemangioblastoma.

Background: A 40-year-old male presented with a complaint of sudden onset diminution of vision in the left eye for 2 weeks. He was a follow-up case with retinal hemangioblastoma in both eyes. He underwent two sittings of fundus fluorescein angiography-guided trans-pupillary thermotherapy 2 years back. Since then, he was regularly followed up for 2 years with stable vision and stable retinal findings. At present, the best-corrected visual acuity (BCVA) in the right eye is 6/6, and in the left eye, it is counting fingers 2 meters. On fundus examination, he had one active hemangioblastoma in the right eye and total retinal detachment in the left eye with multiple active lesions. The right eye was treated with a single sitting of thermotherapy, and the left eye underwent pars plana vitrectomy and angioma excision, followed by silicone oil tamponade. The immediate and late post-operative periods were uneventful, with successful anatomical and functional outcomes. The left eye BCVA on late follow-up was 6/36, no further treatment was advised, and the patient was kept under follow-up and observed closely. Purpose: : To educate regarding the systemic workup, diagnosis, and surgical management of complicated retinal detachment in retinal hemangioblastoma. Synopsis: : Systemic workup, diagnosis, and surgical steps in the management of complicated retinal detachment in retinal hemangioblastoma were performed. Highlights: : Close follow-up, keen observation, and prompt treatment in the early stages of the disease are indispensable to prevent untoward sequelae of retinal hemangioblastoma. A thorough systemic workup is necessary to diagnose the systemic involvements early. Surgery, if indicated for the retinal hemangioblastoma or its associated sequelae, should be performed diligently and with careful handling of blood vessels and anomalous tissues. Online Video Link: https://youtu.be/CkoqWEnaPB8.

Self-Induced Bilateral Retinal Detachments and Traumatic Cataracts in a Patient With Bohring-Opitz Syndrome.

A 9-year-old female with a history of Bohring-Opitz syndrome (BOS), Down syndrome, and autism initially presented with bilateral cataracts and a total retinal detachment in her left eye secondary to chronic self-injurious behavior. The authors report the first case of self-induced retinal detachment and traumatic cataracts in a patient with BOS. For patients who present with self-injurious behavior, the authors advocate for behavioral modifications at home, including the use of "no-no's," supplemental medication if necessary, and behavioral therapy to reduce the risk of self-induced visual injury. The authors also suggest the use of 25-gauge vitrectomy with silicone oil for retinal detachment repair. Finally, given the high risk of irreversible vision loss from amblyopia and recurrent retinal detachments in children with BOS and self-injurious behavior, the authors recommend regular 2-month interval ophthalmic follow-up. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:400-402.].

Optic Nerve Sheath Fenestration for Treatment of Retrolaminar Silicone Oil Migration.

The authors report the case of a 75-year-old man with preexisting glaucoma and recurrent retinal detachment who underwent intraocular silicone oil placement OD resulting in subsequent retrolaminar silicone oil migration to the optic chiasm and vision loss OS. MRI showed silicone oil tracking posteriorly along the right optic nerve to the chiasm. He was placed on high-dose corticosteroids and underwent a successful optic nerve sheath fenestration with improvement of vision in the contralateral eye. Clinicians should be cognizant of the potential for translaminar posterior migration of intraocular silicone oil, as well as the utility of optic nerve sheath fenestration to decompress the anterior visual pathways and restore vision.

Vitrectomy with silicone oil tamponade for retinal detachment associated with giant retinal tears: Favourable outcomes without adjuvant scleral buckling.

PURPOSE: To evaluate the outcomes of pars plana vitrectomy with silicone oil tamponade in the management of retinal detachment associated with giant retinal tears. METHODS: We reviewed 45 eyes of 42 patients with primary retinal detachment associated with giant retinal tears over 10 years at a tertiary referral centre. Patients underwent 23-gauge vitrectomy without adjuvant scleral buckling by a single surgeon and had follow-up at least 6 months after silicone oil removal. RESULTS: Mean follow-up was 37 ± 35 months. Seven eyes (16%) had grade C proliferative vitreoretinopathy, and 16 (36%) had a giant retinal tear ≥180° at baseline. The primary reattachment rate was 84%, and the overall final anatomical success rate was 98%. The mean Snellen visual acuity equivalent at the final visit was 20/58. Final visual acuity ≥20/40 was achieved in 64%. The mean duration of silicone oil tamponade was 10.5 ± 4 weeks. By the final visit, silicone oil had been removed from 44 eyes (98%). CONCLUSION: The high rates of anatomical and functional success support management of giant retinal tears-associated retinal detachment with vitrectomy without adjuvant scleral buckling. Removal of silicone oil at the earliest possible time helps to avoid complications such as keratopathy, glaucoma and visual loss without apparent reason.

Signal Detection Between Fluoroquinolone Use and the Risk of Rhegmatogenous Retinal Detachment: Sequence Symmetry Analysis Using Nationwide South Korean Healthcare Database Between 2004 and 2015.

BACKGROUND AND OBJECTIVES: The association between fluoroquinolone and rhegmatogenous retinal detachment (RRD) has been controversial as a result of inconsistent findings. We set out to detect a possible association of fluoroquinolone use and risk of RRD, using sequence symmetry analysis (SSA). METHODS: We conducted an SSA, case-only design, using a Korean nationwide healthcare database between 2004 and 2015. Exposure was defined as new fluoroquinolone use and outcome as an incident RRD, defined by a diagnosis of RRD (ICD-10: "H33.0") or surgery for RRD. Pairs of exposure and RRD within a 1-year time-window were included. The sequence ratio (SR) was calculated by the ratio of the number of patients prescribed with exposure first and diagnosed with RRD second divided by the number of patients diagnosed with RRD first and prescribed with exposure second. SR was adjusted (aSR) for underlying trends and 95% confidence intervals (CIs) were calculated. In order to observe whether the estimated ratio stabilized over time, we did repeated time-sequential analyses with the cumulative data starting from the 3-year period 2004-2006 to 2015. RESULTS: Fluoroquinolone use had a greater association with RRD as compared with other antibiotics [fluoroquinolone: 5234 pairs; aSR = 1.70 (95% CI 1.61-1.80), first-generation cephalosporin: 4139 pairs; aSR = 1.39 (95% CI 1.31-1.80), second-generation cephalosporin: 5914 pairs; aSR = 1.31 (95% CI 1.24-1.38), third-generation cephalosporin: 3650 pairs; aSR = 0.88 (95% CI 0.83-0.95), extended-spectrum penicillin: 4823 pairs; aSR = 1.29 (95% CI 1.31-1.47), macrolides: 4115 pairs; aSR = 1.31 (95% CI 1.24-1.39)]. Time-sequential analyses supported the association between fluoroquinolone and RRD. CONCLUSIONS: Our detection suggests a possible association between fluoroquinolone use and RRD. However, possible overestimation and reverse causality bias may have influenced our findings due to the limitation of an SSA design.

[Hereditary vitreous degeneration muddy: report of ten cases].

Hereditary vitreous degeneration muddy is rare in clinic. Here we report ten cases (thirteen eyes) of hereditary vitreous degeneration muddy from two families. All patients presented with vitreous opacity, and the textures appeared tough and tensile. Two cases had concurrent detachment of rhegmatogenous retina. HE staining showed red changeableness, and methyl violet staining appeared purple. All patients received vitrectomy with traditional Chinese medicine treatment, and got satisfactory efficacy.

Serous detachment of the macula associated with advanced glaucomatous cupping.

Enhanced depth imaging of the optic nerve of a patient with a serous detachment and profound cupping of the optic nerve secondary to angle-closure glaucoma revealed a large dehiscence of the lamina cribrosa. Adjacent to the defect in the lamina were cystoid spaces within the nerve appearing to contain fluid. The most temporal of these could be seen to extend through the optic nerve and up into the macula. This visualized pathway suggests that mechanical dehiscence of the lamina may allow fluid, possibly derived from cerebrospinal fluid, to track up into the macula.

Optical coherence tomography and histopathology of macular uveitis.

PURPOSE: Spectral-domain optical coherence tomography (SD-OCT) is a noninvasive technique that can provide high-resolution images of macular morphology. The purpose of this study was to examine the pathological mechanism of uveitis and compare the changes in the macula of uveitis patients and the histopathological features of experimentally induced uveitis in mice. METHODS: Macular OCT was performed on 78 eyes of 51 patients of the Eye Hospital of Shandong University of Traditional Chinese Medicine, China, with apparent uveitis changes. C57BL/6 mice were injected with interphotoreceptor retinoid-binding protein (IRBP)-specific T cells from naïve mice immunized with complete Freund adjuvant IRBP(1-20) to induce uveitis. The disease was monitored by indirect fundoscopy. The eyes of the mice with experimental autoimmune uveitis (EAU) were enucleated 18 days after injection and classified according to pathological characteristics. RESULTS: The characteristics of uveitis were classified into six categories. Macular edema was detected in 48 eyes (61.5%); macular epiretinal membrane in 22 eyes (28.2%); choroidal neovascularization and macular lamellar holes in 4 eyes (5.1%), respectively; macular atrophy in 10 eyes (12.8%); and serous neuroepithelium detachment in 22 eyes (28.2%). As in human patients, pathological examinations of mouse EAU showed inflammation, folds, and atrophy of the outer part of the neuroretina, choroidal neovascularization with hemorrhagic retinal detachment, serous neuroepithelium detachment, and epiretinal membrane formation. CONCLUSIONS: Macular OCT of uveitis patients can display different morphological characteristics. Mouse EAU can simulate human uveitis. The comparative analysis of macular OCT in human uveitis and transfer EAU histopathology changes could provide important information on the pathogenesis of human uveitis.

Ozurdex (a slow-release dexamethasone implant) in proliferative vitreoretinopathy: study protocol for a randomised controlled trial.

BACKGROUND: Proliferative vitreoretinopathy (PVR) is the commonest cause of late anatomical failure in rhegmatogenous retinal detachment. Visual and anatomical outcomes remain poor despite advances in vitreoretinal surgical techniques with reported primary failure rates of up to nearly 50%. Numerous adjunctive medications have been evaluated in clinical trials with no agent gaining widespread acceptance and use.This study was designed to investigate the benefits of using a slow-release dexamethasone implant delivered intra-operatively in patients undergoing vitrectomy surgery for retinal detachment with established PVR. METHODS/DESIGN: For the study, 140 patients requiring vitrectomy surgery with silicone oil for retinal detachment with established PVR will be randomised to receive either standard treatment or study treatment in a 1:1 treatment allocation ratio. Both groups will receive the standard surgical treatment appropriate for their eye condition and routine peri-operative treatment and care, differing only in the addition of the supplementary adjunctive agent in the treatment group. The investigated primary outcome measure is stable retinal reattachment with removal of silicone oil without additional vitreoretinal surgical intervention at 6 months. DISCUSSION: This is the first randomised controlled clinical trial to investigate the use of an adjunctive slow-release dexamethasone implant in patients undergoing vitrectomy surgery for retinal detachments with proliferative vitreoretinopathy. TRIAL REGISTRATION: EudraCT No: 2011-004498-96.

Management of retinal detachment in block related globe perforation with pneumatic retinopexy.

Retinal detachment after ocular perforation related to local anesthesia is a common complication, which is usually associated with a poor prognosis despite complex vitreoretinal surgical procedures. We report a case of 62-year-old male with cataract surgery done 4 weeks back with nasal retinal detachment with a posterior break. Pneumatic retinopexy was performed and laser barrage of the breaks was done the next day when the retina got attached. A vision of 20/30 was achieved at the end of 2 months. To the best of our knowledge, this is a first case report in literature where pneumatic retinopexy was used to manage a retinal detachment caused by block-related perforation.

Treatment of serous macular detachment associated with circumscribed choroidal hemangioma.

PURPOSE: To evaluate the effects of transpupillary thermotherapy and intravitreal bevacizumab injection on serous macular detachment and cystoid macular edema (CME) associated with circumscribed choroidal hemangioma. DESIGN: Retrospective, interventional case series. METHODS: We reviewed the records of 12 patients with circumscribed choroidal hemangioma treated with transpupillary thermotherapy and/or intravitreal injection of bevacizumab. We assessed changes in best-corrected visual acuity (BCVA), central foveal thickness by optical coherence tomography, and resolution of serous macular detachment and CME. RESULTS: Six of 8 patients treated with transpupillary thermotherapy showed complete resolution of serous macular detachment and CME and the median minimal angle of resolution (logMAR) BCVA improved from 0.85 to 0.35 (P = .026). Among these 6 patients, 1 had no recurrence for 86 months and 5 had sustained resolution of serous macular detachment for a mean duration of 32.8 months before recurrence. Among the 9 patients treated with bevacizumab (including 5 patients who had transpupillary thermotherapy as a primary treatment), 5 showed resolution of serous macular detachment and the median logMAR BCVA improved from 0.7 to 0.5 (P = .042). Among these 5 patients, 3 had sustained resolution for a mean duration of 5.7 months and 2 showed recurrent serous macular detachment after 3 and 12 months. CONCLUSION: Transpupillary thermotherapy and intravitreal bevacizumab appear effective in the management of symptomatic circumscribed choroidal hemangioma, although recurrence of serous macular detachment and CME developed after long-term follow-up of transpupillary thermotherapy, and the duration of treatment effectiveness appears to be short with bevacizumab.

Transpupillary thermotherapy for circumscribed choroidal hemangioma: clinical profile and treatment outcome.

BACKGROUND AND OBJECTIVE: To report the outcome of treating circumscribed choroidal hemangioma (CCH) using transpupillary thermotherapy (TTT). PATIENTS AND METHODS: An infrared diode laser was used at 810 nm with a median spot size of 2.5 mm, power of 550 mW, and duration of 6.5 minutes; the median number of sittings was two. TTT spared the center of the macula when CCH involved the fovea. RESULTS: The mean age was 36 years (range: 13 to 63 years). CCH was subfoveal in 37.5% of the eyes and exudative retinal detachment was evident in all eyes. The tumor dimensions included thickness (median: 4.05 mm; range: 2.2 to 9.1 mm), horizontal diameter (median: 11 mm; range: 6 to 17.7 mm), and vertical diameter (median: 9.4 mm; range: 5.9 to 15.8 mm). After TTT, visual acuity improved in 44%, was maintained in 37%, and worsened in 19% of eyes. Likewise, tumor regression was achieved in thickness, vertical, and horizontal diameter in proportion of 20%, 15%, and 11%, respectively. The exudative retinal detachment resolved in 94% of eyes at the last follow-up. The median follow-up was 9.5 months. CONCLUSION: Macula-sparing TTT was effective in causing regression of CCH and resorption of associated exudative retinal detachment, and helped in preserving or improving vision in 81% of treated eyes.

Heavier-than-water silicone oil mixture as a long-term tamponade agent: a pilot study.

PURPOSE: To evaluate the tamponade effect on the retina of a heavier-than-water silicone oil mixture and to compare it with the effect of silicone oil. METHODS: Prospective, non-randomised, comparative pilot study. Phakic/pseudophakic patients with retinal detachment undergoing vitrectomy with Densiron 68 or silicone oil were recruited. The 'separation volume', defined as the relative volume of the space between intraocular tamponade agent and retina, was estimated using magnetic resonance imaging in both groups and compared. RESULTS: Nine participants were included; 4 received silicone oil and 5 Densiron 68. The mean separation volume was statistically significantly larger in the silicone oil group (0.477 ± 0.419 cm(3)) than in the Densiron group (0.042 ± 0.013 cm(3); p = 0.014). CONCLUSIONS: In this study Densiron achieved an excellent tamponade effect in the retina.

Peripapillary schisis with serous detachment in advanced glaucoma.

PURPOSE: To describe features associated with the development and resolution of peripapillary retinoschisis with an underlying serous detachment in a patient with primary open angle glaucoma. This presentation occurred in the absence of an observed optic nerve coloboma, congenital, or acquired optic nerve head pit. CASE REPORT: A patient with advanced glaucomatous optic nerve cupping developed a temporally localized peripapillary serous detachment in the right eye which spontaneously resolved. RESULTS: Optical coherence tomography demonstrated an area of retinoschisis with underlying serous detachment contiguous with the temporal disc margin. Although fluorescein angiography was not performed and the presence of a peripapillary subretinal neovascular membrane could not be ruled out, an atypical coloboma, optic nerve head pit, or peripapillary subretinal neovascular membrane was not observed during biomicroscopy or scanning laser ophthalmoscopy. The retinoschisis and detachment resolved without intervention. CONCLUSION: Peripapillary retinoschisis with an underlying serous detachment may develop in subjects with advanced glaucoma. Although the occurrence of the findings in this case may be unrelated to glaucomatous optic neuropathy, the likelihood that a pathogenic mechanism linked to advanced glaucoma may be responsible for the development of peripapillary schisis and serous detachment should alternatively be taken into consideration. This case documents its spontaneous resolution without intervention.

[Basic principles and clinical application of retinal laser therapy]. / Grundlagen und klinische Anwendung der Lasertherapie an der Netzhaut.

The scientific background of laser photocoagulation of the ocular fundus was studied extensively by several investigators in the 1970 s and 1980 s. The basic principles were successfully resolved during that time and clinical consequences for proper application of the laser photocoagulation for various diseases were deduced. The present paper gives an overview about the physical basics of laser-tissue interactions during and after retinal laser treatment and the particular laser strategies in the treatment of different retinal diseases. Thus, it addresses the issue of the impact on tissue of laser parameters as wavelength, spot size, pulse duration and laser power. Additionally, the different biological tissue reactions after laser treatment are presented, such as, e. g., for retinopexia or macular treatments as well as for diabetic retinopathies. Specific laser strategies such as the selective laser treatment of the RPE (SRT) or the transpupillary thermotherapy (TTT) are presented and discussed.

Macular schisis and detachment associated with presumed acquired enlarged optic nerve head cups.

PURPOSE: To describe a clinical syndrome of macular schisis and detachment in patients with acquired optic nerve head cupping resulting from glaucoma. DESIGN: Retrospective review of five patients. METHODS: Patients were included if they had optic nerve cupping and macular schisis with or without detachment with no other identifiable cause. The patients had to have no leakage on fluorescein angiography and no vitreous traction on examination or on optic coherence tomography (OCT). These patients were followed up and visual acuity, intraocular pressure, and the findings of serial fundus and OCT examinations were noted. RESULTS: Five patients had schisis with or without detachment of the macula with pronounced optic nerve head cupping. One patient had resolution of the macular fluid after filtering surgery for uncontrolled glaucoma. Two patients underwent a vitrectomy with intraocular gas and had almost total resolution of macular fluid and improved vision. CONCLUSIONS: Macular schisis and detachment can occur in patients with presumed enlarged optic nerve head cups in the absence of obvious congenital anomalies of the disk. The authors believe the cause is leakage of fluid from the vitreous through a tiny hole in the thin tissue of the cup. This is a similar mechanism to that seen in patients with optic pits. A vitrectomy or steps to reduce the intraocular pressure may result in resolution of the fluid and improved vision.

Leptospirosis and panuveitis in a dog.

A case of panuveitis as a result of leptospiral infection in an 8-year-old Jack Russell Terrier is reported. Mild hyphema, aqueous flare, and partial serous retinal detachments were noted in both eyes on initial ophthalmic examination. The serum microscopic agglutination testing for Leptospira serovarieties revealed titers of 1 : 12 800 for Leptospira canicola and 1 : 200 for Leptospira icterohemorrhagiae. With medical therapy, the panuveitis resolved and functional vision was regained. Four weeks after the initial presentation, convalescent titers were 1 : 200 for L. canicola and 1 : 100 for L. icterohemorrhagiae. The incidence of leptospirosis, along with difficulties diagnosing, and current medical therapies for this disease are also discussed.