Surgical Outcomes of Retinal Detachment in Knobloch Syndrome.

PURPOSE: To describe the rate, characteristics, and outcomes of rhegmatogenous retinal detachment (RD) in patients with Knobloch syndrome. DESIGN: A single-center retrospective cohort study. PARTICIPANTS: Fifty patients with Knobloch syndrome diagnosed clinically, with or without molecular confirmation of recessive pathogenic COL18A1 variants. METHODS: A retrospective chart review of all patients diagnosed with Knobloch syndrome from November 1, 1983 to March 31, 2023. Demographic data, ophthalmic evaluation at baseline and follow-up, interventions, and final anatomic and visual outcomes were collected. MAIN OUTCOME MEASURES: Rate, time of onset, characteristics, and treatment outcomes of RD. RESULTS: Fifty patients with Knobloch syndrome were included. Males constituted 56% of cases. The diagnosis was confirmed with molecular genetic testing in 37 (74%) patients. Twenty-two patients (44%) had documented occipital bony defects or scalp lesions. Forty-eight of 100 eyes (48%) developed RD at a mean (standard deviation [SD]) age of 6.5 (6.1) years. The mean (SD) follow-up was 7.7 (5.6) years (range, 6 months to 24.3 years). Macular hole-related RD comprised 33% of RD cases. The overall single-surgery success rate was 36% and the final anatomic success rate was 70%. Macular hole-related RD carried a slightly worse prognosis with a 58% final anatomic success rate. Vitrectomy with adjunct scleral buckle and silicone oil tamponade provided the highest single-surgery success (62.2%). In eyes with measurable best-corrected visual acuity (BCVA), the mean preoperative BCVA was 1.2 logarithm of the minimum angle of resolution (Snellen equivalent, 20/320). After successful repair, mean visual acuity was 1.3 logarithm of the minimum angle of resolution (Snellen equivalent, 20/500). CONCLUSIONS: Retinal detachment in Knobloch syndrome is frequent and occurs in young children. Macular hole-related RD comprises one third of RD cases and requires careful macular evaluation. Vitrectomy, combined with scleral buckling and silicone oil tamponade, appears to provide the best anatomic outcomes. FINANCIAL DISCLOSURES: The authors have no proprietary or commercial interest in any materials discussed in this article.

Early-Onset Myopia and Retinal Detachment without Typical Microcoria or Severe Proteinuria due to a Novel LAMB2 Variant.

PURPOSE: To describe the ocular and renal features, as well as outcomes of retinal detachment repair, in patients with a novel, homozygous laminin ß-2 (LAMB2) pathogenic variant. DESIGN: Single-center retrospective chart review of patients with a homozygous variant, c.619T>C p.(Ser207Pro), in the LAMB2 gene. SUBJECTS: Eleven patients (22 eyes) from 4 families. METHODS: Demographic data and ocular findings were recorded. Patients were recalled for a detailed renal evaluation. MAIN OUTCOME MEASURES: Ocular features, renal features, and outcomes of retinal detachment repair. RESULTS: The mean age at presentation was 6.0 (range, 1-26) years. None of the study eyes had microcoria, and none of the patients had nephrotic-range proteinuria. The mean refraction and axial length were -7.9 diopters (range, -4.0 to -12.0 diopters) and 25.3 (range, 22.7-27.7) mm, respectively. Eleven eyes (50%) had cataract at presentation. Fifteen eyes had a clear view to the fundus and all showed tessellated myopic fundus, avascular peripheral retina evident clinically or on fluorescein angiography, and rudimentary fovea. Optic disc pallor was observed in 10 eyes (66.7%). Straightened retinal vessels, abnormal vascular emanation (situs inversus) from the optic disc, supernumerary vascular branching at the optic disc, and vascular tortuosity were observed in 10 (66.7%), 2 (13.4%), 2 (13.4%), and 2 (13.4%) eyes, respectively. Discrete areas of punched-out chorioretinal atrophy were observed in 4 (26.7%) eyes. Spectral-domain OCT showed retinal and choroidal thinning in 13 eyes (86.7%), retinoschisis temporal to the fovea in 2 eyes (13.4%), and rudimentary fovea in 15 eyes (100%). Among the 22 eyes, 14 eyes (63.6%) developed rhegmatogenous retinal detachment (RRD), mostly during childhood, of which 5 patients had bilateral RRD. Eight eyes were operated on and 6 (75%) achieved retinal reattachment at the last follow-up. The mean preoperative visual acuity was 20/300 and the mean postoperative visual acuity at the last follow-up was 20/400. CONCLUSIONS: This study describes a distinct phenotype of LAMB2-related disease with a novel, homozygous LAMB2 variant, and further expands the spectrum of ophthalmic and renal features, and the molecular genetic basis, of LAMB2-related disease. Because the typical microcoria and nephrotic-range proteinuria might be absent, the retinal features can guide the diagnosis. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any materials discussed in this article.

Combined central retinal vein occlusion and cilioretinal artery occlusion as the initial presentation of frosted branch angiitis: a case report and literature review.

PURPOSE: To report a case of combined central retinal vein occlusion (CRVO) with cilioretinal artery occlusion (CLRAO) that heralded the development of frosted branch angiitis (FBA). CASE REPORT: A 25-year-old healthy male presented with sudden painless visual loss in his left eye with a visual acuity (VA) of 20/300. Fundus exam and fluorescein angiography showed signs of combined CRVO and CLRAO. Without treatment, his vision gradually improved until it reached 20/30 within four months. Five months after initial presentation, he returned with severe visual loss (20/400) in the same eye and a clinical picture of severe occlusive periphlebitis resembling a frosted branch angiitis pattern associated with severe macular edema. This was promptly and successfully treated with systemic steroids and immunosuppressive medications. CONCLUSION: CRVO in young population can have an unusual course and one should carefully rule out underlying uveitic etiologies in each visit. Clinical suspicion and close follow­up are required for early detection and timely management of FBA.

LEPREL1 -RELATED GIANT RETINAL TEAR DETACHMENTS MIMIC THE PHENOTYPE OF OCULAR STICKLER SYNDROME.

PURPOSE: To describe the features of retinal detachments and high myopia in patients with novel pathogenic variants in LEPREL1 and report a possible association with nephropathy. METHODS: Retrospective study of 10 children with biallelic LEPREL1 pathogenic variants. Data included ophthalmic features, surgical interventions, and genetic and laboratory findings. RESULTS: 10 patients (8 females) from three families with homozygous (2) or compound heterozygous (1) variants in LEPREL1 were included. At presentation, mean age was 9.9 ± 2.6 years. Mean axial length was 28.9 ± 1.9 mm and mean refraction was -13.9 ± 2.8 diopters. Bilateral posterior subcapsular cataracts were present in eight patients (80%), with lens subluxation in five eyes of three patients (30%). Rhegmatogenous retinal detachments (RRD), associated with giant retinal tears (GRT), developed in seven eyes of five patients (50%) at a mean age of 14.14 ± 5.9 years. Six were successfully reattached with mean Snellen best-corrected visual acuity improving from 20/120 preoperatively to 20/60 at last follow-up. Urinalysis in nine patients revealed microhematuria and/or mild proteinuria in six patients (67%). CONCLUSION: LEPREL1 -related high myopia confers a high risk of early-onset GRT-related RRD. The ocular phenotype may be confused with that of ocular Stickler syndrome if genetic testing is not performed. Further investigations into a potential association with renal dysfunction are warranted.

Saudi Arabian retinopathy of prematurity national telemedicine program: Achievements and challenges.

The Saudi Arabian Retinopathy of Prematurity National Telemedicine programme (SAROP) is a product of the National Committee for Retinopathy of Prematurity (ROP). The program includes ROP telescreening, diagnosis, and management of cases requiring treatment. Digital retinal images and filled ROP software requests were uploaded from 20 level-3 neonatal intensive care units (NICU) in the Kingdom of Saudi Arabia (KSA) to the King Khaled Eye Specialist Hospital server and the ROP telemedicine website. The data were accessed and reported by qualified retinal and pediatric ophthalmologists. Currently, retinal wide-angle digital cameras are available in 20 of the 31 level-3 NICUs of the Ministry of Health, Kingdom of KSA. This telemedicine approach is practical and effective in detecting and managing ROP cases. In the first 2.5 years, encouraging results were noticed with no unfavorable outcomes in the participating NICUs. Technical challenges were resolved promptly to ensure that the program ran smoothly. Therefore, this ideal state-of-the-art ROP telemedicine program could be also applied to similar and neighboring countries.

Loss of the collagen IV modifier prolyl 3-hydroxylase 2 causes thin basement membrane nephropathy.

The glomerular filtration barrier (GFB) produces primary urine and is composed of a fenestrated endothelium, a glomerular basement membrane (GBM), podocytes, and a slit diaphragm. Impairment of the GFB leads to albuminuria and microhematuria. The GBM is generated via secreted proteins from both endothelial cells and podocytes and is supposed to majorly contribute to filtration selectivity. While genetic mutations or variations of GBM components have been recently proposed to be a common cause of glomerular diseases, pathways modifying and stabilizing the GBM remain incompletely understood. Here, we identified prolyl 3-hydroxylase 2 (P3H2) as a regulator of the GBM in an a cohort of patients with albuminuria. P3H2 hydroxylates the 3' of prolines in collagen IV subchains in the endoplasmic reticulum. Characterization of a P3h2ΔPod mouse line revealed that the absence of P3H2 protein in podocytes induced a thin basement membrane nephropathy (TBMN) phenotype with a thinner GBM than that in WT mice and the development of microhematuria and microalbuminuria over time. Mechanistically, differential quantitative proteomics of the GBM identified a significant decrease in the abundance of collagen IV subchains and their interaction partners in P3h2ΔPod mice. To our knowledge, P3H2 protein is the first identified GBM modifier, and loss or mutation of P3H2 causes TBMN and focal segmental glomerulosclerosis in mice and humans.

Pediatric Retinal Detachment in Homozygous Protein C Deficiency: Genetic and Phenotypic Description of a Single Family.

Homozygous protein C deficiency is a rare hypercoagulability disorder. This study describes the ocular manifestations and the genetic background in a family with two affected children. This is a retrospective review of ophthalmic examinations, investigations, genetic testing, and blood work-up of two children with homozygous protein C deficiency from a single family. A family with a positive history of consanguineous marriage was found to have two affected children with homozygous protein C deficiency. Abnormal visual behavior was the presenting symptom. Both children had bilateral total tractional retinal detachments at presentation. Skin manifestations included episodes of discoloration and bruising. Laboratory work-up revealed absent protein C activity. Genetic testing confirmed the presence of a homozygous pathogenic mutation in protein C gene (NM_000312.3: c.1297G>A: p.Gly433Ser). Homozygous protein C deficiency should be considered in the differential diagnosis of early-onset tractional retinal detachment in infancy. Although rare, the ophthalmologist may be the first to encounter the condition, and treatment with protein C replacement or anticoagulants may be life-saving. Examination under anesthesia with fluorescein angiography and laser treatment early in life may be warranted to preserve vision. [Ophthalmic Surg Lasers Imaging Retina. 2022;53:293-296.].

Vitrectomy versus Vitrectomy with Scleral Buckling in the Treatment of Giant Retinal Tear Related Retinal Detachments: An International Multicenter Study.

PURPOSE: To determine the practice pattern for treating giant retinal tear (GRT) related detachments, and their anatomic and visual outcomes with pars plana vitrectomy (PPV) with or without scleral buckling (SB). DESIGN: Retrospective cohort study. SUBJECTS: Eyes with GRT detachments repaired from 2008 to 2020 with at least 6 months of follow-up from 7 institutions in North and South America, Europe, and Asia. METHODS: Eyes repaired using PPV versus PPV/SB were compared. MAIN OUTCOME MEASURES: Anatomic and functional outcomes. RESULTS: A comparable number of eyes underwent PPV (n = 101) and PPV/SB (n = 99). Except for history of developmental abnormalities, prior intraocular surgery, and lens status, no differences in baseline demographics, ocular characteristics, or intraoperative surgical adjuncts were observed. The overall single surgery anatomic success (SSAS) at 6 months and 1 year were similar between the groups (82.2% and 77.2% of PPV, and 87.9% and 85.7% of PPV/SB). When stratified by age, the 1-year SSAS rate was higher for PPV/SB (88.5%) than PPV (56.3%) (P = 0.03) for children <18 years. For both children and adults, the mean best-corrected visual acuity (BCVA) at baseline did not differ between the PPV and PPV/SB groups. However, for children, mean BCVA at 1 year was better in the PPV/SB than PPV groups (P = 0.001) while for adults, no difference was found between the 2 groups. The mean time to the first redetachment was 7.9 months in the PPV group and 5.5 months in the PPV/SB group (P = 0.8). Proliferative vitreoretinopathy was the most common cause for redetachment (70.4% of PPV and 93.8% of PPV/SB in redetached eyes; P = 0.1). Postoperative complications were also similar between the 2 groups, including ocular hypertension, epiretinal membrane, and cataract formation. CONCLUSIONS: PPV and PPV/SB are equally popular among surgeons globally for managing GRT detachments and have comparable anatomic and visual outcomes in adults. In children, PPV/SB is superior to PPV for anatomic and functional success at 1 year. In adults, the relief of traction by the GRT may reduce peripheral traction and obviate the need for an SB. However, in children, a supplemental SB can be beneficial as complete vitreous shaving and posterior hyaloid detachment, and postoperative positioning, are difficult in this group.

Internal Limiting Membrane Folds as a Presenting Sign in Acute Initial-Onset Vogt-Koyanagi-Harada Disease: A Case Report.

PURPOSE: To report a case of initial-onset acute Vogt-Koyanagi-Harada (VKH) presenting with internal limiting membrane (ILM) folds as the initial clinical sign. METHODS: This is a retrospective case review. RESULTS: A 36-year-old woman with a recent history of periorbital injection of cosmetic filler who presented with sudden blurred vision in the right eye of a few hours duration. Initial fundus examination revealed only ILM folds in the right eye. Next day, the patient developed the full ophthalmic clinical picture of initial-onset acute VKH disease. Multimodal imaging showed features of ILM undulations and acute VKH disease. Uveitis was treated successfully with systemic corticosteroids combined with mycophenolate mofetil. The patient had complete resolution of posterior segment inflammation and exudative retinal detachment. CONCLUSION: ILM folds/undulations can be one of the early signs in acute VKH disease, and may serve as an important early clinical clue for this diagnosis.

Adjunctive Intravitreal Triamcinolone Acetonide for Exudative Retinal Detachment in Coats Disease.

Purpose: This work aims to assess the value of intravitreal triamcinolone acetonide (IVTA) as an adjunctive therapy in advanced Coats disease with exudative retinal detachment (ERD). Methods: A retrospective review was conducted of patients with Coats disease stage 3 or higher who received IVTA to decrease subretinal fluid (SRF), facilitate retinal ablative therapy, and avoid surgical drainage. Primary outcomes were SRF resolution and avoidance of surgical SRF drainage. Results: Seventeen eyes of 17 patients (mean, [SD] age, 3.9 [3.4] years) met the inclusion criteria. ERD configuration was bullous in 7 and shallow in 10 eyes. Following a single IVTA injection, ablative therapy was achieved after a mean (SD) of 2.1 (3.0) weeks. Complete SRF resolution was observed in 13 eyes (76.4%) after a mean of 1.3 IVTA injections and a mean of 2 (SD, 1.27) laser sessions, and none of these eyes required SRF drainage up to last follow-up (mean [SD], 50.5 [26.24] months). In 4 eyes with bullous ERD at presentation, SRF persisted (P = .015) despite additional measures including surgical drainage. Final visual acuity ranged from 20/100 to no light perception. Cataract developed in 12 of the 17 eyes (70.5%). None developed an increase in intraocular pressure at final follow-up. Conclusions: IVTA injection can be a helpful adjunctive modality to address SRF in advanced Coats disease. It may obviate the need to surgically drain SRF to effectively treat the condition, particularly when the ERD is not highly bullous.

Multimodal Imaging, Tele-Education, and Telemedicine in Retinopathy of Prematurity.

Retinopathy of prematurity (ROP) is a disease that affects retinal vasculature in premature infants and remains one of the leading causes of blindness in childhood worldwide. ROP screening can encounter some difficulties such as the lack of specialists and services in rural areas. The evolution of technology has helped address these issues and led to the emergence of state-of-the-art multimodal digital imaging devices such fundus cameras with its variable properties, optical coherence tomography (OCT), OCT angiography, and fluorescein angiography which has helped immensely in the process of improving ROP care and understanding the disease pathophysiology. Computer-based imaging analysis and deep learning have recently been demonstrating promising outcomes in regard to ROP diagnosis. Telemedicine is considered an acceptable alternative to clinical examination when optimal circumstances for ROP screening in certain areas are lacking, and the expansion of these programs has been reported. Tele-education programs in ROP have the potential to improve the quality of training to physicians to optimize ROP care.

Comparison of heads up three dimensional visualization system to conventional microscope in retinopathy of prematurity related tractional retinal detachment.

To report the outcomes, advantages and disadvantages of a heads-up three-dimensional (3D) visualization system compared to the conventional microscope in pediatric tractional retinal detachment (TRD) surgery secondary to advanced stage retinopathy of prematurity (ROP). Medical records of patients with ROP stage 4 or 5 who underwent surgery for tractional retinal detachment at King Khaled Eye Specialist Hospital between September 2017 and July 2019 were identified and reviewed. Eyes were divided into 2 groups, eyes that underwent surgery with a 3D heads-up platform (3D group) and eyes that underwent surgery with a conventional microscope (conventional group). Data were collected on neonatal history, visual acuity, intraoperative complications and success rates between groups.Eighteen eyes of 14 patients who underwent surgical repair of TRD related to ROP. Postoperative outcomes were compared between 10 eyes (7 patients) in the 3D group and 8 eyes (7 patients) in the conventional group There was no statistically significant difference in success rate between both groups (75% conventional group vs 70% 3D group). Partial or complete reattachment was achieved in 7 eyes in 3D group compared to 6 eyes in conventional group. Lower postmenstrual age at the time of the first surgery and presence of retinal breaks were associated with poorer surgical outcome. Heads up 3D visualization system is feasible in tractional retinal detachment related to ROP with similar success rate and no increased risk of complications when compared to conventional microscope. This system may be advantageous in advanced pediatric tractional retinal detachment surgeries.

Comparison of Posterior Hyaloid Assessment Using Preoperative Optical Coherence Tomography and Intraoperative Triamcinolone Acetonide Staining During Vitrectomy.

PURPOSE: To compare the specificity of diagnosing posterior vitreous detachment (PVD) using preoperative optical coherence tomography (OCT) versus intraoperative triamcinolone acetonide (TA) staining in patients undergoing vitrectomy. PATIENTS AND METHODS: This retrospective cohort study included patients undergoing pars plana vitrectomy for diverse retinal pathologies. Intraoperatively, surgeons evaluated the posterior hyaloid status with TA staining and compared it with preoperative OCT findings. RESULTS: One hundred six patients underwent intraoperative assessments of posterior hyaloid status, with 72% (76/106) of the eyes showing positive staining. Sixty-two patients had also undergone preoperative OCT. Of the patients diagnosed with PVD on preoperative OCT, 50% (15/30) showed positive TA staining intraoperatively. The sensitivity of preoperative OCT assessment was 83.3%, and its specificity was 65.9%. CONCLUSION: Preoperative OCT imaging is associated with lower sensitivity and specificity for diagnosing PVD when compared to intraoperative TA staining.

Pitfalls of Advanced Retinopathy of Prematurity Presentation: A Content Analysis of Medical Records.

PURPOSE: To identify the pitfalls in retinopathy of prematurity (ROP) screening leading to advanced disease at Ministry of Health (MOH) hospitals in Saudi Arabia. METHODS: A qualitative deductive content analysis was used to study the phenomena of defects in ROP screening. A retrospective review of medical records of newborns presenting to two tertiary eye care centers with advanced ROP (stage 4 and 5) from January 2012 to June 2019 was completed. An extensive review of the original files at the referring hospitals was conducted, including the general condition and findings of ophthalmic examination and the sequence of follow-up until the infant was discharged/referred. Data analysis was completed using pre-structured categorization matrix. RESULTS: Records of 29 infants with advanced stage ROP were identified. Only 13 medical records were available and obtained. The pitfalls in screening found in the study were failure to refer by the neonatologist, delayed follow-up by the ophthalmologist, failure to follow-up by the ophthalmologist, failure to diagnose by the ophthalmologist, poor documentation in patient files, unavailability of ophthalmologist, family negligence, lack of treatment and delayed referral to a higher center, and progression despite timely screening and management. CONCLUSION: Although clear ROP screening guidelines are available, implementations of these guidelines are suboptimal. This study showed that the most common defect in screening is physician's inadequacy and unavailability. A proper network of competent ROP screening physicians in all neonatal intensive care units should be established. Centers for advanced ROP surgery should be allocated to deliver a timely surgical care if needed.

Pemphigus Vulgaris-Associated Anterior Scleritis: A Case Study.

Ocular involvement in pemphigus vulgaris (PV) is relatively rare. The conjunctiva and eyelids are considered the most common affected sites in ocular pemphigus. Scleritis is rarely reported as a manifestation of PV. We present a case report of anterior scleritis as a manifestation of PV and its response to rituximab therapy.

Development of neovascular glaucoma after intraocular surgery in Pierson syndrome.

Purpose: To report a patient with Pierson syndrome who presented with neovascular glaucoma (NVG) after cataract surgery.Methods: Retrospective case report.Results: A 17-year old monocular female presented with sudden onset of pain and decreased vision in the right eye. On examination, she had intraocular pressure (IOP) of 50 mmHg, aggressive iris neovascularization (NVI) and 3-piece IOL. Fundus examination revealed pale disc with tessellated fundus and parapapillary atrophy. Vascular arcades were vertically stretched with avascular ischemic retina starting from the near periphery. Macula appeared thin and atrophic. An intravitreal injection of 0.05 mg/0.1 ml bevacizumab was given to the right eye followed by Ahmed glaucoma valve (AGV) implantation. Assessment of her brother revealed similar posterior segment changes. A subsequent urine analysis showed proteinuria and high albumin to creatinine ratio. Next-generation sequencing for LAMB2 gene revealed a homozygous c.4573 + 1 G > A variant confirming the diagnosis of Pierson syndrome.Conclusion: This case expands our knowledge on retinal ischemia in the setting of Pierson syndrome. Close monitoring after intraocular surgery is recommended to look for the development of NVG.

Evolution of macular hole in enhanced S-cone syndrome.

PURPOSE: To describe the stages of development and natural course of a full-thickness macular hole (FTMH) in a patient with enhanced S-cone syndrome (ESCS). METHODS: This study reported the serial ophthalmologic examinations and macular spectral-domain optical coherence tomography (SD-OCT) imaging over a period of 6 years in a 29-year-old man with ESCS confirmed by electroretinography (ERG) and NR2E3 molecular genetic analysis. RESULTS: At presentation, patient had night blindness and visual acuity (VA) of 20/300 in the right eye (OD) and 20/100 in the left eye (OS). Examination showed bilateral retinal midperipheral pigmentary deposits and a macular schisis in OD. Electroretinography and NR2E3 genetic analysis confirmed ESCS. A year later, a lamellar MH (LMH) appeared at the fovea in OD. SD-OCT confirmed it as inner retinal layer LMH with outer retinal preservation and displayed, on the temporal side of the LMH, prominent splitting between the inner and outer retinal layers. At 2 years, a focal defect in the ellipsoid zone appeared on SD-OCT, followed by split in the outer retinal layer creating a progressively expanding outer LMH. The latter had rolled edges which then fused with the inner LMH margins creating a single full-thickness FTMH. Over the next 4 years, enlargement of the FTMH with increased adjacent retinal splitting continued. No visible vitreous abnormalities or vitreoretinal traction forces were identified at any stage during follow-up. VA OD remained unchanged. CONCLUSION: This case illustrates that the clinical evolution of FTMH in ESCS may be progressive and likely involves degeneration and intraretinal, rather than vitreoretinal, traction. This should be kept in mind when considering surgical intervention in these cases.

Persistent subretinal fluid following diabetic tractional retinal detachment repair: risk factors, natural history, and management outcomes.

PURPOSE: To study the natural history, anatomical and functional outcomes of persistent subretinal fluid (SRF) after pars plana vitrectomy (PPV) for diabetic tractional retinal detachment (TRD) and combined traction-rhegmatogenous retinal detachment (TRRD). METHODS: Retrospective interventional case series of 43 patients (46 eyes) with persistent SRF following PPV for diabetic TRD or combined TRRD from January 2010 to December 2017 at single tertiary institution. Primary outcomes included best corrected visual acuity (BCVA) and central foveal thickness (CFT). RESULTS: Thirty-one eyes (67.4%) had macula-off TRD, 5 (10.9%) had fovea-threatening TRD and 10 (21.7%) had combined TRRD. The mean (± SD) duration of decreased vision was 48.0 ± 58.2 weeks. The mean follow-up duration was 21 ± 13.2 months. Residual macular SRF was detected by optical coherence tomography in all eyes at 3 months and in 10 eyes (23.8%) at 12 months after surgery. Only 3 eyes (6.5%) had persistent SRF at final follow up. The mean time to resolution was 10.6 ± 4.1 months [range 6.0-23.0]. Thirteen eyes received additional intervention to address SRF. The mean CFT gradually improved until final follow-up (P-value < 0.001). The mean BCVA improved from 1.62 ± 0.88 LogMAR at presentation to 1.05 ± 0.76 LogMAR at final follow up. No statistically significant difference in final BCVA was found between eyes that had intervention and eyes that were observed (P value = 0.762). CONCLUSION: Persistent SRF after diabetic vitrectomy resolves slowly over time with gradual improvement in visual acuity. Additional drainage of persistent SRF may not be necessary.

Posterior Segment Characterization in Children With Pierson Syndrome.

BACKGROUND AND OBJECTIVE: Pierson syndrome is a rare genetic disease defined by congenital nephrotic syndrome in association with microcoria. The authors aim to describe the posterior segment and retinal features in Pierson syndrome. PATIENTS AND METHODS: A retrospective chart review of nine patients diagnosed with Pierson syndrome was ascertained. Details of ophthalmic history, ocular examination, retinal imaging, and surgical interventions were obtained during a median duration of 17 months of follow-up (range: 6 to 60 months). Retinal interventions included scatter laser photocoagulation and surgical retinal repair. RESULTS: Sixteen eyes of nine patients were included. The axial length of five eyes with flat retina was 26.59 mm ± 0.99 mm. Highly myopic features including tessellated fundus with accompanying optic disc pallor, unidentifiable cup, and abnormal retinal vascular emanation from the disc were observed in all eyes (100%), whereas 12 eyes (75%) had parapapillary chorioretinal atrophy. Features of abnormal retinal vascularization included avascular peripheral retina on fluorescein angiography, aberrant course of the temporal arcades in 13 eyes (81.3%), and straightened nasal retinal blood vessels in 12 eyes (75%). Tortuous retinal blood vessels were observed in three eyes (18.75%). Surgical repair was performed in five out of seven eyes with rhegmatogenous retinal detachment (RRD). Recurrence was observed in all eyes, which required two to three procedures to achieve final reattachment. CONCLUSIONS: Combined features of high axial myopia with incomplete peripheral vascular maturation characterize the posterior segment in Pierson syndrome. Careful posterior segment examination is essential to detect RRD or retinal neovascularization. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:618-627.].