Joyce N. Mbekeani

Investigation of antioxidant systems in human meibomian gland and conjunctival tissues

Oxidative stress (OS) associated with direct contact with the environment and light exposure is a very potent and continuous stressor of the ocular surface and internal structures of the eye that are required to manage its effects. Constant replenishment of tears together with the superficial lipid layer produced by the meibomian glands (MG) is one protective mechanism. The lipid-rich fraction of the tears coats the deeper aqueous fraction, preventing its evaporation. However, lipids are particularly sensitive to oxidative damage that could alter tear film quality. To counteract oxidative damage, MG along with other structures of the ocular surface use primary antioxidant (AO) systems to limit OS damage such as lipid peroxidation. Limited information concerning the primary enzymatic AO system of the human MG prompted this investigation. Using different approaches (RT-PCR, enzymatic activity assays and immuno-fluorescent microscopy), we determined the presence, distribution and subcellular locations of the major AO enzymes belonging to the classical catalytic triad (superoxide dismutase, catalase and glutathione peroxidases) in adult human MG and conjunctiva (Conj). We showed that both tissues exhibit glutathione peroxidase expression. In addition to the ubiquitous cytosolic GPx1 protein, there was significant expression of GPx2, GPx4 and GPx7. These isoforms are known to preferentially scavenge phospholipid-hydroperoxide compounds. This characterization of the primary AO system of human MG and Conj may help pave the way for the development of diagnostic procedures and have implications for treatment of common MG dysfunction (MGD) and dry eye syndrome (DES).

Corrigendum to ‘Visual Pathway Injuries in Pediatric Ocular Trauma – A Survey of the National Trauma Data Bank from 2008-2014’ [Pediatric Neurology 85 (2018) 43-50]

Corrigendum to ‘Visual Pathway Injuries in Pediatric Ocular Trauma A Survey of the National Trauma Data Bank from 2008-2014’ [Pediatric Neurology 85 (2018) 43-50] Ryan Gise , Timothy Truong, Afshin Parsikia , Joyce N. Mbekeani a,d a Department of Ophthalmology & Visual Sciences, Montefiore Medical Center/Albert Einstein College of Medicine, Bronx, New York b Montefiore Medical Center/Albert Einstein College of Medicine, Bronx, New York c Department of Surgery (Trauma), Jacobi Medical Center, Bronx, New York d Department of Surgery (Ophthalmology), Jacobi Medical Center, Bronx, New York

Late Recognition of a Case of Oculo-Auriculo-Vertebral Spectrum.

Purpose To report the late recognition of a case oculo-auriculo-vertebral spectrum (OAVS). Case Report A 14-year-old male patient with a history of spinal scoliosis and anal atresia presented with a right ocular growth that was increasing in size and causing irritation. He was discovered to have a right limbal dermoid and forniceal lipodermoid. All visual functions were normal and the rest of the anterior and posterior segments were unremarkable. External examination revealed facial asymmetry and ipsilateral hemifacial microsomia and a pre-auricular skin tag. A diagnosis of oculo-auriculo-vertebral spectrum (OAVS) or Goldenhar syndrome was made. Conclusions Growth of a limbal dermoid helped identify oculo-auriculo-vertebral spectrum, a disorder with a wide phenotypic spectrum that can affect all systems. Early recognition and syndromic designation is imperative; full pediatric survey of suspected cases will help rule out the life-threatening manifestations of the spectrum.

Morning Glory Syndrome with Carotid and Middle Cerebral Artery Vasculopathy.

Purpose To report a case of incidental asymptomatic atypical morning glory syndrome (MGS) with concomitant ipsilateral carotid and middle cerebral dysgenesis. Case Report A 6-year-old child was discovered to have incidental findings of MGS, with atypia. All visual functions were normal including vision and stereopsis. Neuroimaging revealed ipsilateral carotid and middle cerebral vascular narrowing without associated collateral vessels or cerebral ischemia commonly seen in Moyamoya disease. Subsequent annual examinations have been stable, without signs of progression. Conclusions This case demonstrates disparity between structural aberrations and final visual and neurological function and reinforces the association between MGS and intracranial vascular disruption. Full ancillary ophthalmic and neuroimaging studies should be performed in all patients with MGS with interval reassessments, even when the patient is asymptomatic and functionally intact.

Answer: An incidental large arachnoid cyst associated with compensated hydrocephalus in a patient with hemophilia A

tp://dx.doi.org/10.1016/j.ijpam.20 52-6467/Copyrighta 2015, King Fais Elsevier B.V. This is an open acces MRI of the brain was obtained to better define the cystic lesion and adjacent structures impacted by its mass effects. Additional 3-dimension FIESTA sequences (fast imaging employing steady-state acquisition) were employed to obtain strong hyperintensity signals of fluid-filled tissues (Fig. 3A). These tests revealed a large lobulated sellar and suprasellar cystic lesion, most likely an arachnoid cyst. This

What is your Diagnosis? Hydrocephalus in a patient with hemophilia A

tp://dx.doi.org/10.1016/j.ijpam.20 52-6467/Copyright a 2015, King Fais Elsevier B.V. This is an open acces Figure 1 Sagittal CT scan of the brain showing an irregular sellar and suprasellar cystic lesion (white arrow), dilated lateral ventricles and normal fourth ventricle (red arrow) and a patent aqueduct of Sylvius (blue arrow). A 17-year-old male with a past history of hemophilia A presented to the emergency department with headaches, dizziness and an unsteady gait following a recent fall. He had intercurrent hemophilia arthropathy, and his medications were tramadol and anti-hemophilic factor VIII. A CT scan revealed hydrocephalus with a patent aqueduct of Sylvius, a normal fourth ventricle and an irregular cystic enlargement in the region of the third ventricle as well as a dilatation of the lateral ventricles (Fig. 1). There were no intracranial hemorrhages noted. Ophthalmology was consulted to rule out papilledema. Ophthalmic examination revealed an alert and oriented young man with bilateral visual acuities of 20/25, Ishihara colour vision of 15/15 OU, normal pupillary reactions without relative afferent defects and normal anterior segments. Mild

Infantile orbital myofibroma

a Dept. of Surgery, North Bronx Health Network, Bronx, NY, USA b Dept. of Ophthalmology and Visual Sciences, Albert Einstein College of Medicine of Yeshiva University, Bronx, NY, USA c Service d’Histopatology, CHU Clermont Ferrand, France d Image-Guided Clinical Neurosciences and Connectomics, IGCNC, Université d’Auvergne, Clermont Ferrand, France e Dept. of Ophthalmology, CHU Clermont Ferrand, Clermont-Ferrand, France f Dept. of Ophthalmology, KFSH&RC, Riyadh, Saudi Arabia