CENTRAL RETINAL VEIN OCCLUSION IN 12-YEAR-OLD GIRL WITH METHYLENETETRAHYDROFOLATE REDUCTASE MUTATION: A CASE REPORT AND REVIEW OF THE LITERATURE.

PURPOSE: This case report describes a central retinal vein occlusion in a healthy 12-year-old girl who developed retinal neovascularization at 24 years of age. To the knowledge of the authors, this is the longest time between a reported pediatric central retinal vein occlusion event and neovascularization. METHODS: The patient underwent a full history, physical examination, and laboratory workup to determine potential risk factors contributing to the vascular event. Fundus photographs, optical coherence tomography, and fluorescein angiography were performed throughout the patient's treatment course. RESULTS: Family history was noncontributory, but laboratory testing revealed a mildly elevated homocysteine level and homozygous C677T mutation in methylenetetrahydrofolate reductase. As a result, she was started on folate supplementation. The patient has had no further ocular or systemic thrombotic events to date. CONCLUSION: Pediatric patients presenting with central retinal vein occlusion should undergo a systemic workup and require long-term follow-up to avoid complications, such as intraocular hemorrhage, tractional retinal detachments, and neovascular glaucoma.

Haptic Erosion Following Sutureless Scleral-fixated Intraocular Lens Placement.

PURPOSE: To describe the clinical features and visual outcomes of eyes with conjunctival haptic erosion after sutureless intrascleral (SIS) fixated intraocular lens (IOL) placement. DESIGN: Retrospective case series. SUBJECTS: Patients experiencing haptic erosion after SIS fixation between January 1, 2013, and March 1, 2022. METHODS: A multicenter, multisurgeon, retrospective review. MAIN OUTCOME MEASURES: Clinical features, visual outcomes, and treatment options following haptic erosions after SIS fixation. RESULTS: Nineteen eyes with haptic erosion were identified. The mean age at initial SIS fixation was 64 ± 12 years (range, 38-81 years). There were 5 (26%) eyes with a history of conjunctiva involving ocular surgery, including scleral buckle surgery and tube shunt surgery. Trocar-assisted fixation was performed in 15 (79%) eyes, whereas needle fixation was used in 4 (21%) eyes. Eighteen (95%) sets of haptics were flanged with a low temperature cautery. Seventeen (90%) sets of haptics were externalized superiorly and inferiorly, and 2 (10%) sets of haptics were externalized nasally and temporally. Haptics were covered by conjunctiva in 14 (74%) eyes and by scleral flap in 5 (26%) eyes. All patients experienced a single haptic erosion, of which 8 (43%) were located superiorly, 9 (47%) inferiorly, and 2 (10%) temporally. The mean interval between the initial SIS fixation and haptic erosion was 278 ± 437 days. After correction of the erosion, 18 (95%) eyes had a stable IOL at the last follow-up, with no recurrence of haptic erosion. In this series, there were no cases of endophthalmitis. CONCLUSIONS: Haptic erosion is a notable complication after SIS fixated IOL surgery but may be repaired with favorable visual outcomes. Careful evaluation of the conjunctiva should be considered before the surgery. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Acute-onset postoperative endophthalmitis after cataract surgery performed by resident and attending physicians at a university teaching hospital.

PURPOSE: To evaluate acute-onset postoperative endophthalmitis following cataract surgery and to compare rates between resident and attending physician-performed cohorts. SETTING: Bascom Palmer Eye Institute, Miami, Florida. DESIGN: Retrospective chart review. METHODS: Records of patients diagnosed with endophthalmitis within 6 weeks of surgery performed by attending and resident physicians between January 1, 2015, and December 31, 2020, were reviewed. Total cataract volume was obtained from institutional records, and resident case totals were obtained from case logs. Endophthalmitis cases were obtained from billing records and confirmed with chart review. RESULTS: There were 22 cases of endophthalmitis among 32 505 cases (0.068%). Endophthalmitis occurred in 6 of 6447 (0.093%) resident cases and 16 of 26 058 (0.061%) attending cases ( P = .55). The most common bacterial isolates were coagulase-negative Staphylococcus (8/22, 36.3%) and Streptococcus species (3/22, 13.6%), with negative cultures in 10 (10/22, 45.5%). Initial treatment with vitreous tap and injection of intravitreal antibiotics was performed in 21 eyes (21/22, 95.4%) and vitrectomy with intravitreal antibiotic injection in one (1/22, 4.5%). Vitrectomy was performed secondarily in 9 patients (9/22, 40.9%). Corrected distance visual acuity (CDVA) at last follow-up was ≥20/40 in 13 eyes (13/22, 59%) and ≤hand motions in 3 eyes (3/22, 13.6%). CDVA (logMAR mean ± SD) was 1.22 ± 1.16 in resident and 0.49 ± 0.79 in attending cases ( P = .11). CONCLUSIONS: In the current study, acute-onset postoperative endophthalmitis developed infrequently following cataract surgery. The rates and visual outcomes of endophthalmitis were similar in resident and attending cases.

Retinal Folds as a Clinical Feature of X-Linked Retinoschisis: A Series of Three Cases.

BACKGROUND AND OBJECTIVE: The most common clinical features of X-linked retinoschisis (XLRS) include macular schisis in a spoke wheel pattern and peripheral schisis, though other findings such as vitreous veils, vascular attenuation, and subretinal fibrosis have been described. This is the first report to describe retinal folds as a characteristic feature in patients with XLRS. PATIENTS AND METHODS: This was a case series of patients presenting to the retina service at Bascom Palmer Eye Institute with genetically confirmed XLRS. Patients included in this report underwent examination under anesthesia with multimodality imaging. RESULTS: Three patients with XLRS were found to have retinal folds, including a newly characterized "retinal scroll" seen on examination and multimodality imaging. CONCLUSIONS: The presence of a retinal fold should yield a differential diagnosis that includes XLRS in the correct clinical context. Panel-based genetic testing and multimodal imaging are useful in guiding clinical management. [Ophthalmic Surg Lasers Imaging 2022;53(6):326-331.].

Widefield Fluorescein Angiography in the Fellow Eyes of Patients with Presumed Unilateral Persistent Fetal Vasculature.

PURPOSE: To examine the retinal vascular findings on widefield fluorescein angiography (FA) in the fellow eyes of patients with unilateral persistent fetal vasculature (PFV). DESIGN: Retrospective case series. PARTICIPANTS: Consecutive patients with unilateral PFV evaluated by a single physician at an academic medical center from February 1, 2011, to November 30, 2018. METHODS: Clinical and demographic information, including age, gender, race, ethnicity, affected eye, subtype, stalk origin, complications on presentation, length of follow-up, and examination findings, was reviewed using the electronic medical record. Fluorescein angiograms of the affected and fellow eyes were reviewed by 2 authors for characteristic retinal vascular abnormalities. Agreement between the authors' scores was analyzed using Cohen's Kappa. MAIN OUTCOME MEASURES: Fluorescein angiography abnormalities, including peripheral vessel avascularity, aberrant circumferential vessels, terminal supernumerary branching, regional capillary dropout, terminal bulbing, abnormal choroidal flush, abnormal vessel straightening, and peripheral vessel leakage or dilation. RESULTS: Inclusion criteria were met by 41 patients. The average age at initial visit was 10.0 months. The average length of follow-up was 36.4 months. Abnormalities on FA were seen in 31 (75.6%) fellow eyes: peripheral vessel avascularity in 27.5 (67.1%), aberrant circumferential vessels in 20 (48.8%), terminal supernumerary branching in 17 (41.5%), regional capillary dropout in 9 (22.0%), terminal bulbing in 6 (14.6%), abnormal choroidal flush in 3.5 (8.5%), and abnormal vessel straightening and peripheral vessel leakage in 2.5 (6.1%). Statistical analysis disclosed an overall observed agreement of 93.4% among the raters (κ = 0.84, P < 0.0001). CONCLUSIONS: Retinal vascular abnormalities seen in patients with unilateral PFV were present in the majority of fellow eyes. This suggests that unilateral PFV may in fact be a bilateral, asymmetric process, but the clinical significance of these subtle findings is not known.

Ocular trauma secondary to exercise resistance bands during the COVID-19 pandemic.

OBJECTIVE: To characterize injuries caused by exercise resistance bands. METHOD: Single-site retrospective case series of patients presenting to the Bascom Palmer Eye Institute emergency room with ocular injuries secondary to exercise resistance bands from March through September 2020. RESULTS: Eleven patients (9 males, 2 females, 14 eyes) were reviewed. Eight patients had a unilateral injury (3 right eyes, 5 left eyes) while 3 had bilateral injuries. Iritis was the most common presentation, seen in all 11 patients, followed by hyphema (9 patients, 82%), and vitreous hemorrhage (4 patients, 36%). Among affected eyes, the mean presenting visual acuity was approximately 20/100, improving to 20/40 on the last follow up (p = 0.06). However, 4 eyes (33%) had vision ≤20/60 at last follow up. CONCLUSIONS: Exercise resistance bands can cause a wide spectrum of ocular injuries, some leading to long-term vision loss. As such, we recommend that patients strongly consider using eye protection goggles or glasses while using resistance bands for exercise.

Intraocular Invasion of Ocular Surface Squamous Neoplasia Through a Corneal Wound.

PURPOSE: We report a case of intraocular invasion of ocular surface squamous neoplasia (OSSN) through a cataract surgery wound that manifested as an anterior chamber membrane, and perform a review of the literature. METHODS: An 87-year-old woman with history of an incompletely excised OSSN 10 days prior presented with decreased vision due to an anterior chamber membranous film that seemed connected to an old cataract wound. The membrane was biopsied, and histopathology revealed dysplastic squamous epithelial cells of conjunctival origin. Ten days later the tumor expanded to cover the entire iris surface, and a modified enucleation was performed. RESULTS: Histopathology revealed invasive OSSN extending deep to the ciliary body and laterally into the anterior chamber. The patient developed a submandibular node metastasis 7 months later. Literature review revealed six cases of invasive OSSN resulting from extension through a corneal wound, with varied presentations and outcomes. CONCLUSION: Our case is the first to report invasive OSSN presenting as an anterior chamber membrane as a result of incomplete excision of an ocular surface malignancy adjacent to a corneal wound poses a risk for intraocular tumor extension and distant metastasis.

Genotypic and Phenotypic Antibiotic Resistance in Staphylococcus Epidermidis Endophthalmitis.

BACKGROUND AND OBJECTIVE: To evaluate antibiotic resistance patterns in Staphylococcus epidermis endophthalmitis isolates, identify antibiotic resistance genes, and compare this to their phenotypic resistance. MATERIALS AND METHODS: Retrospective case series of S. epidermidis isolates from January 2012 to December 2017. Phenotypic resistance was determined from minimum inhibitory concentration values. Genotypic resistance was determined from DNA microarray. The relationship between these was analyzed using Cohen's kappa and predictive value. RESULTS: Seventy-five isolates were included. More than 60% were resistant to methicillin and erythromycin. Approximately 60% possessed the resistance genes for methicillin and erythromycin, and 25% for clindamycin. There was near perfect agreement between genotype and phenotype for erythromycin and methicillin (kappa = 0.88 and 0.89, respectively). Approximately 98% (P < 0.0001) of isolates with erythromycin and methicillin resistance genes and 90% (P = 0.0006) with clindamycin resistance genes were phenotypically resistant to those antibiotics. None were resistant to vancomycin. CONCLUSION: The relationship between genotypic and phenotypic antibiotic resistance highlights that resistance may be polygenic, and genes may be silent. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:S13-S16.].

A unique case of coats plus syndrome and dyskeratosis congenita in a patient with CTC1 mutations.

Coats plus syndrome (CP) is a rare condition characterized by bilateral exudative retinal telangiectasias with associated systemic disorders primarily affecting the brain, bone and gastrointestinal tract due to a mutation in the CTC1 gene. CTC1 mutations are also known to cause dyskeratosis congenita (DC), which is an inherited bone marrow failure syndrome characterized by skin pigmentation abnormalities, nail dystrophy, and oral leukoplakia. This is the first reported case of a patient diagnosed with both CP and DC caused by compound heterozygous CTC1 gene mutations. Moreover, one of the variant mutations found in this patient has never been published before.

Select pediatric vitreoretinal disease in the setting of Turner's syndrome.

PURPOSE: To report 2 cases of pediatric vitreoretinal disease in the setting of Turner's syndrome. OBSERVATIONS: A 4-year-old girl with Turner's syndrome was referred for evaluation of a tractional retinal detachment in the right eye. Fundoscopic examination disclosed temporal dragging of the macula in the right eye, and vascular nonperfusion in the right and left eyes. Genetic testing revealed a novel frameshift mutation in the LRP5 gene consistent with familial exudative vitreoretinopathy (FEVR). The patient was treated with laser. A 14-year-old girl with Turner's syndrome presented with nyctalopia. Dilated fundus exam disclosed peri-foveal pigmentary changes and peripheral bone spicules. Full-field electroretinography demonstrated decreased rod and cone responses, consistent with retinitis pigmentosa (RP). CONCLUSIONS AND IMPORTANCE: Vitreoretinal disease, including RP and FEVR, is rarely observed in patients with Turner's syndrome.

Lipomatous Atrial Septal Hypertrophy: A Review of Its Anatomy, Pathophysiology, Multimodality Imaging, and Relevance to Percutaneous Interventions.

Lipomatous atrial septal hypertrophy (LASH) is a histologically benign cardiac lesion characterized by excessive fat deposition in the region of the interatrial septum that spares the fossa ovalis. The etiology of LASH remains unclear, though it may be associated with advanced age and obesity. Because of the sparing of the fossa ovalis, LASH has a pathognomonic dumbbell shape. LASH may be mistaken for various tumors of the interatrial septum. Histologically, LASH is composed of both mature and brown (fetal) adipose tissue, but the role of brown adipose tissue remains unclear. In interventional procedures requiring access to the left atrium, LASH may interfere with transseptal puncture, as traversing the thickened area can reduce the maneuverability of catheters and devices. This may cause the needle to enter the epicardial space, causing dangerous pericardial effusions. LASH was once considered a contraindication to percutaneous device closure of atrial septal defects because of an associated increased risk for incorrect device deployment. However, careful attention to preprocedural imaging and procedural intracardiac echocardiography enable interventional cardiologists to perform procedures in patients with LASH without serious complications. In this review article, the authors describe anatomic and functional aspects of LASH, with emphasis on their roles in percutaneous interventions.

The role of multimodality imaging in percutaneous left atrial appendage suture ligation with the LARIAT device.

Atrial fibrillation (AF), the most common cardiac arrhythmia, is a significant cause of embolic stroke. Although systemic anticoagulation is the primary strategy for preventing the thromboembolic complications of AF, anticoagulants carry major bleeding risks, and many patients have contraindications to their use. Because thromboembolism typically arises from a clot in the left atrial appendage (LAA), local therapeutic alternatives to systemic anticoagulation involving surgical or percutaneous exclusion of the LAA have been developed. Surgical exclusion of the LAA is typically performed only as an adjunct to other cardiac surgeries, thus limiting the number of eligible patients. Furthermore, surgical exclusion of the LAA is frequently incomplete, and thromboembolism may still occur. Percutaneous LAA exclusion includes two approaches: transseptal delivery of an occlusion device to the LAA and epicardial suture ligation of the LAA, the LARIAT procedure. In the LARIAT procedure, a pretied snare is placed around the epicardial surface of the LAA orifice via pericardial access. Proper snare placement is achieved with epicardial and endocardial magnet-tipped guidewires. The endocardial wire is advanced transvenously to the LAA apex after transseptal puncture. The epicardial wire, introduced into the pericardial space, achieves end-to-end union with the endocardial wire at the LAA apex. The snare is then placed over the LAA, tightened, and sutured. On the basis of early clinical experience, the LARIAT procedure has a high success rate of LAA exclusion with low risk for complications. The authors describe the indispensable role of real-time transesophageal echocardiography in the guidance of LAA epicardial suture ligation with the LARIAT device.