Endothelial Dysfunction in Systemic Sclerosis.

Systemic sclerosis, commonly known as scleroderma, is an autoimmune disorder characterized by vascular abnormalities, autoimmunity, and multiorgan fibrosis. The exact etiology is not known but believed to be triggered by environmental agents in a genetically susceptible host. Vascular symptoms such as the Raynaud phenomenon often precede other fibrotic manifestations such as skin thickening indicating that vascular dysfunction is the primary event.

Voltage sensor dynamics of a bacterial voltage-gated sodium channel NavAb reveal three conformational states.

High-resolution structures of voltage-gated sodium channels (Nav) were first obtained from a prokaryotic ortholog NavAb, which provided important mechanistic insights into Na selectivity and voltage gating. Unlike eukaryotic Navs, the NavAb channel is formed by four identical subunits, but its ion selectivity and pharmacological profiles are very similar to eukaryotic Navs. Recently, the structures of the NavAb voltage sensor at resting and activated states were obtained by cryo-EM, but its intermediate states and transition dynamics remain unclear.

Contemporary Outcomes After Partial Resection of Infected Aortic Grafts.

Introduction: Aortic graft infection remains a considerable clinical challenge, and it is unclear which variables are associated with adverse outcomes among patients undergoing partial resection.

Methods: A retrospective, multi-institutional study of patients who underwent partial resection of infected aortic grafts from 2002 to 2014 was performed using a standard database. Baseline demographics, comorbidities, operative, and postoperative variables were recorded.

Microcornea and Thickened Lens in Angle Closure following Nonsurgical Treatment of Retinopathy of Prematurity.

Purpose: To characterize the clinical features in young patients with angle closure and to determine the characteristics associated with acquired anterior segment abnormality following retinopathy of prematurity (ROP) treatment.

Methods: We performed two retrospective case-control series. In the first series, we identified consecutive young angle closure patients without prior surgeries, with and without a history of ROP treatment; in the second series we identified consecutive patients who underwent ROP treatment, without and without anterior segment changes.

Subretinal Injection of Voretigene Neparvovec-rzyl in a Patient With RPE65-Associated Leber's Congenital Amaurosis.

Leber's congenital amaurosis (LCA) is a rare inherited retinal degeneration (IRD) that causes severe vision loss, nyctalopia, and nystagmus within the first few years of life. RPE65 gene mutations cause approximately 6% of LCA cases and have become the target for therapy since voretigene neparvovec-rzyl became the first U.S.

Differentiating Veins From Arteries on Optical Coherence Tomography Angiography by Identifying Deep Capillary Plexus Vortices.

Purpose: To introduce a simple method for differentiating retinal veins from arteries on optical coherence tomography angiography (OCTA).

Design: Cross-sectional pilot study.

Methods: Four default en face slabs including color depth encoded, grayscale full-thickness retina, superficial plexus, and deep capillary plexus (DCP) from nine 3×3-mm and nine 6×6-mm OCTA scans were exported and aligned.

Vitreoretinal lymphoma followed by systemic diffuse large B cell lymphoma.

Vitreoretinal lymphoma as the presenting diagnosis in association with a systemic lymphoma without central nervous system involvement is exceedingly rare, and the classification of this condition is not well-established. Here, we describe a patient with intermittent blurry vision in the left eye for 2 years in the setting of a recent incidental diagnosis of diffuse large B cell lymphoma from an axillary lymph node biopsy. The diagnosis of panuveitis with an extensive exudative retinal detachment was made.

Circumferential retinal hemorrhages after ophthalmic examination with scleral depression in an infant with anti-VEGF treated retinopathy of prematurity.

We report a case of retinal hemorrhages in a baby with retinopathy of prematurity (ROP) following examination with indirect ophthalmoscopy and scleral depression. There have been rare reports of examination-induced retinal hemorrhages during ROP screening, although those hemorrhages were diffusely scattered in the posterior pole. In this report the hemorrhages were found on the surface of the neovascular ridge.

An Eight-Year-Old Girl Presents With Two Weeks of Vision Loss and Nightly Headaches.

Intraocular medulloepithelioma is a rare congenital tumor that arises from the nonpigmented epithelium of the ciliary body. It is the second most common primary intraocular neoplasm during the first decade of life. It may present with an iris mass or cyst (56%), glaucoma (48%), cataract (26%), leukocoria (18%), decrease in vision (41%), or pain (30%).

Clinical Features, Antibiotic Susceptibilities, and Treatment Outcomes of Endophthalmitis Caused by Staphylococcus epidermidis.

Purpose: To report the clinical features, antibiotic susceptibilities, and visual acuity outcomes with endophthalmitis caused by methicillin-sensitive Staphylococcus epidermidis and methicillin-resistant S. epidermidis.

Design: Retrospective case series.

A Novel Pathogenic Variant in NDP Gene With Incomplete Penetrance Manifests as X-Linked Familial Exudative Vitreoretinopathy.

Familial exudative vitreoretinopathy (FEVR) is a rare hereditary ocular disorder characterized by incomplete or abnormal development of peripheral retinal vasculature. The genes responsible for this disorder are associated with the wingless-related integration site (Wnt) signaling pathway, a critical pathway for the development of normal retinal vasculature. A pathogenic variant in any one of these genes may disrupt retinal vasculogenesis.

Microcornea, posterior megalolenticonus, persistent fetal vasculature, chorioretinal coloboma (MPPC) syndrome: Case series post vitrectomy.

Purpose: MPPC syndrome has been described as a syndrome that presents with chorioretinal coloboma, posterior megalolenticonus, persistent fetal vasculature, and chorioretinal coloboma. The purpose of our study is to report three patients who present with a variation of MPPC syndrome who each underwent pars plana vitrectomy, pars plana lensectomy, and amblyopic management. Clinical characteristics, ancillary test findings, and post-surgical functional results are compared to what is reported in the literature.

Comparison of Athletes and Nonathletes Undergoing Thoracic Outlet Decompression for Neurogenic Thoracic Outlet Syndrome.

Background: Neurogenic thoracic outlet syndrome (NTOS) is the most common form of thoracic outlet syndrome (TOS) and may occur from injury, occupational stress, or athletic endeavors. Although most patients with NTOS will improve after first-rib resection and scalenectomy (FRRS), the prognostic risk factors for success remain unclear. Athletes are a very motivated and disciplined demographic and therefore should be a group more likely to respond to FRRS for NTOS than nonathletes.

Management of inadvertent needle penetration resulting in subretinal triamcinolone acetonide and retinal detachment.

Purpose: To report management of inadvertent needle penetration during subtenons triamcinolone acetonide administration resulting in retinal detachment.

Observations: A 71-year-old female with history of diabetes, hypothyroidism, and mild myopia underwent subtenons triamcinolone acetonide (TA) injection in the right eye for nodular scleritis. There was unexpected patient movement concurrent with the injection resulting in needle penetration, subretinal and intravitreal injection of TA, superotemporal retinal break, and macula-involving retinal detachment.

Multi-drug resistant scleral buckle infection.

Purpose: To describe a case of Multi-drug resistant scleral buckle infection.

Observations: A 56 year-old male with history of retinal detachment repair with scleral buckle 20 years prior presented with 8 months of intermittent pain and redness in the left eye. The patient was diagnosed with scleral buckle infection, the buckle was removed, and cultures revealed multi-drug resistant The postoperative course included orbital cellulitis treated with systemic linezolid, clarithromycin, and imipenem.

Clinical features, antimicrobial susceptibilities, and treatment outcomes of patients with culture positive endophthalmitis after penetrating keratoplasty.

Purpose: To report the clinical features organisms and treatment outcomes in patients with endophthalmitis after penetrating keratoplasty (PK).

Methods: Retrospective noncomparative case series.

Results: Eleven eyes of 11 patients with culture positive endophthalmitis after PK were included.

Retinal vasproliferative tumor in a case of X-linked retinoschisis detachment.

Purpose: To describe the first published case of X-linked retinoschisis (XLRS) detachment with retinal vasoproliferative tumor (RVPT) and provide a literature review of the subject.

Observations: The authors describe a case of a 17 year old male with X-linked retinoschisis who presented with a retinal detachment and a retinal vasoproliferative tumor. The patient was treated with pars plana vitrectomy, endolaser, subtenon's kenalog and anti-VEGF (vascular endothelial growth factor) intravitreal injections.

Management of Retinopathy of Prematurity--Use of Anti-VEGF Therapy.

Retinopathy of prematurity (ROP) is a leading cause of childhood blindness worldwide. Although laser photocoagulation re-mains the gold standard for treating threshold and prethreshold disease (type 1 ROP), the off-label use of anti‒vascular endothelial growth factor (anti-VEGF) therapy to treat ROP is increasing. Benefits include acute regression of ROP, growth of retinal vasculature beyond the demarcation line, lesser degree of myopia and peripheral visual field loss, and avoidance of sedation and intubation required for laser.

Antiphosphospholipid syndrome presenting with amaurosis fugax and cotton wool spots.

Purpose: To describe the importance of considering vaso-occlusive disease on the differential diagnosis of a patient presenting with amaurosis fugax (AF) and unilateral cotton wool spots (CWS).

Observations: A 69-year-old female with history of obesity, hyperlipidemia and recent orthopedic surgery, presented with 3 days of worsening monocular AF and CWS in the right eye. She was diagnosed with antiphosphospholipid syndrome based on positive serologic testing for antiphosphatidylserine IgM, anticardiolipin IgM.

Comparison of Regional vs. General Anesthesia for Surgical Repair of Open-Globe Injuries at a University Referral Center.

Purpose: This study compares the clinical features and physician selection of either Regional Anesthesia (peribulbar or retrobulbar block) with Monitored Anesthesia Care (RA-MAC) or General Anesthesia (GA) for open globe injury repair.

Design: A non-randomized, comparative, retrospective case series at a University Referral Center.

Participants: All adult repairable open globe injuries receiving primary repair between January 1st, 2004 and December 31st, 2014 (11 years).

Midterm and long-term follow-up in competitive athletes undergoing thoracic outlet decompression for neurogenic thoracic outlet syndrome.

Background: Neurogenic thoracic outlet syndrome (NTOS) results from compression of the brachial plexus by the clavicle, first rib, and scalene muscles and may develop secondary to repetitive motion of the upper extremity. Athletes routinely perform repetitive motions, and sports requiring significant arm and shoulder use may put the participant at increased risk for NTOS. Competitive athletes who develop NTOS may require first rib resection and scalenectomy (FRRS) for symptomatic relief.

Adenoid cystic carcinoma of the lacrimal gland in a 14-year-old male.

Adenoid cystic carcinoma (ACC) of the lacrimal gland is an aggressive, malignant epithelial neoplasm. This tumor is rarely seen in adults and even less commonly seen in children and adolescents; thus, there have been no large studies to date describing the optimal treatment of this malignancy in the pediatric population. Here, we report a case of lacrimal gland ACC in a 14-year-old male treated with neoadjuvant intra-arterial chemotherapy followed by globe-sparing tumor resection and chemoradiation.