Periocular Microcystic Adnexal Carcinoma: A Case Report and a Major Review.

Purpose: To describe a patient with periocular microcystic adnexal carcinoma (MAC) and to review the clinical presentation, systemic work-up, histopathologic features, and outcome of all previously reported periocular MAC.

Methods: A major literature review. PubMed/MEDLINE and Google Scholar databases were searched for all well-documented cases of periocular MAC.

Postoperative diplopia after medial-inferior orbital wall decompression in patients with thyroid eye disease.

10% of patients undergoing MAI decompression for TED developed new-onset diplopia MAI decompression remains an excellent option for orbital decompression for TED.

Cyclin D1 Expression and Molecular Genetic Findings in Periocular Histiocytoses and Neoplasms of Macrophage-Dendritic Cell Lineage.

Purpose: Frequent activating mutations in the mitogen-activated protein kinase (MAPK) pathway genes have been identified in histiocytoses. MAPK signaling consistently upregulates cyclin D1. The goal of this study was to determine whether cyclin D1 expression by immunohistochemistry is a useful diagnostic marker for periocular histiocytoses and to further characterize their genetic basis.

Lacrimal drainage apparatus melanoma remotely following treatment and resolution of conjunctival melanoma.

Lacrimal drainage apparatus melanoma is a rare entity that may arise primarily or, more commonly, as secondary involvement from melanoma originating elsewhere. Conjunctival melanoma may involve the lacrimal drainage apparatus (LDA) via spread along the canalicular epithelium, separate in situ processes, or direct invasion. Only seven cases exist in the literature where conjunctival melanoma remotely recurred in the LDA.

Management of Orbital Masses: Outcomes of Endoscopic and Combined Approaches With No Orbital Reconstruction.

Introduction: The endoscopic endonasal approach to management of orbital pathology has expanded. Due to the rarity of these conditions, most reports in the literature consist of small case reports. We report a series from a single institution with a focus on outcomes.

Combined Endoscopic and External Resection of an Inverted Papilloma of the Lacrimal Sac: A Case Report and Review of the Literature.

Background: Inverted papillomas (IPs) are benign, locally aggressive neoplasms with a high recurrence rate that most commonly arise from the lateral nasal wall. Rarely, IP can originate from the lacrimal sac and/or nasolacrimal duct (NLD) system. A 58-year-old man presented with chronic epiphora and an enlarging mass inferior to his left medial canthal tendon (MCT) for 2 years.

Beyond Traditional Structure-Based Drug Design: The Role of Iron Complexation, Strain, and Water in the Binding of Inhibitors for Hypoxia-Inducible Factor Prolyl Hydroxylase 2.

A combination of structure-based drug design and medicinal chemistry efforts led us from benzimidazole-2-carboxamide with modestly active hypoxia-inducible factor prolyl hydroxylase 2 inhibition to certain benzimidazole-2-pyrazole carboxylic acids that were more potent as well as orally efficacious stimulators of erythropoietin secretion in our in vivo mouse model. To better understand the structure-activity relationship, it was necessary to account for (i) the complexation of the ligand with the active site Fe, (ii) the strain incurred by the ligand upon binding, and (iii) certain key water interactions identified by a crystal structure analysis. With this more complete computational model, we arrived at an overarching paradigm that accounted for the potency differences between benzimidazole-2-carboxamide and benzimidazole-2-pyrazole carboxylic acid enzyme inhibitors.

Endoscopic Management of Lacrimal System Dysgenesis and Dacryocystoceles in Fraser Syndrome: A Case Report and Literature Review.

Background: Fraser syndrome is an autosomal recessive disorder characterized primarily by syndactyly, cryptophthalmos, urinary tract anomalies, ambiguous genitalia, and laryngeal anomalies. A 28-year-old man with Fraser syndrome presented with cryptophthalmos, microphthalmia, lacrimal system dysgenesis, and chronic sinusitis.

Objective: The patients' clinical condition and surgical treatment are described.

Concerns about Quadrupole ICP-MS Lead Isotopic Data and Interpretations in the Environment and Health Fields.

There has been a massive increase in recent years of the use of lead (Pb) isotopes in attempts to better understand sources and pathways of Pb in the environment and in man or experimental animals. Unfortunately, there have been many cases where the quality of the isotopic data, especially that obtained by quadrupole inductively coupled plasma mass spectrometry (Q-ICP-MS), are questionable, resulting in questionable identification of potential sources, which, in turn, impacts study interpretation and conclusions. We present several cases where the isotopic data have compromised interpretation because of the use of only the major isotopes Pb/Pb and Pb/Pb, or their graphing in other combinations.

Orbital T-cell lymphoma versus lupus panniculitits: a T-cell-mediated spectrum of orbital lymphoproliferative disease.

This is a case description of a single male patient found to have T-cell-mediated inflammation and lymphoproliferation of the orbit. Chronic T-cell-mediated inflammatory disease can pose a diagnostic challenge particularly in its differentiation from a neoplastic process. The histopathology in this case demonstrated features of both lupus erythematosus panniculitis and features of orbital T-cell lymphoma.

Basaloid adenocarcinoma of the lacrimal sac: A rare entity and approach to management.

Presented is a rare case of basaloid adenocarcinoma of the lacrimal sac. Low grade features of this malignancy permitted a conservative surgical approach to maximize functional and cosmetic outcomes. This is retrospective case description of a single 77-year-old-female patient who underwent a dacryocystorhinostomy with silicone tubes at an outside hospital for epiphora, but was referred to the Wills Eye Hospital Oculoplastics Department for a lacrimal sac biopsy showing low-grade carcinoma with basaloid features.

A case of concurrent silent sinus syndrome, thyroid eye disease, idiopathic orbital inflammatory syndrome, and dacryoadenitis.

This is a retrospective case description of a single female patient found to have concomitant bilateral silent sinus syndrome, bilateral thyroid eye disease, unilateral dacryoadenitis, and idiopathic orbital inflammation that presented as progressive unilateral right-sided proptosis. The spectrum of inflammatory orbital diseases can make discerning between different entities challenging, but more unique in this case was the simultaneous presentation with bilateral silent sinus syndrome, a rare entity in its own right. Identifying each of these concurrent disease processes is important to establishing a multidisciplinary treatment approach to address all the patient's orbital and peri-orbital pathology.

Paranasal sinus lymphoma: Retrospective review with focus on clinical features, histopathology, prognosis, and relationship to systemic lymphoma.

Background: Paranasal sinus lymphoma is a rare clinical entity.

Methods: We conducted a retrospective case series of 68 patients with biopsy-confirmed paranasal sinus lymphoma with attention on systemic disease association.

Results: Of 63 patients with paranasal sinus lymphoma, 35 (56%) had systemic involvement.

A rare case of orbital hodgkin lymphoma demonstrating therapeutic response to a novel systemic medication.

Orbital Hodgkin lymphoma is infrequent, representing less than 1% of adult orbital malignancies. It is typically seen in the setting of terminally advanced systemic disease. This case highlights orbital, head, and neck disease in a systemically stable individual treated with the novel anti-CD30 monoclonal antibody brentuximab vedotin.

Goat horn-induced intracranial emphysema and orbital injury.

This is a case description of a male patient found to have orbital and intracranial emphysema, specifically with air in his cavernous sinuses bilaterally following penetrating trauma to the medial orbit from a goat's horn. There were no orbital or skull base fractures. Although the presence of traumatic intracranial emphysema is not uncommon, it is typically the result of direct communication of the cranial vault with the paranasal sinuses in the setting of associated fracture or, alternatively, from direct penetration and inoculation.

Glutamatergic stimulation induces GluN2B translation by the nitric oxide-Heme-Regulated eIF2α kinase in cortical neurons.

The activation of N-Methyl D-Aspartate Receptor (NMDAR) by glutamate is crucial in the nervous system function, particularly in memory and learning. NMDAR is composed by two GluN1 and two GluN2 subunits. GluN2B has been reported to participate in the prevalent NMDAR subtype at synapses, the GluN1/2A/2B.

The Effectiveness of a Mobile Clinic in Improving Follow-up Eye Care for At-Risk Children.

Purpose: To investigate the potential of a mobile ophthalmic unit in the schoolyard to improve the follow-up rate for children who have failed an optometric in-school screening program. Previously, the optometric program made referrals to the center and only 53% of students attended the desired ophthalmology consultation.

Methods: This was a cohort study of students conducted in elementary school lots in socioeconomically disadvantaged communities.

Acute Charles Bonnet Syndrome following Hughes procedure.

A 69-year-old male experienced monocular formed visual hallucinations after occlusion of the right eye following resection of eyelid basal cell carcinoma and reconstruction with a Hughes procedure (tarsoconjunctival flap). His symptoms included recurrent, well-defined, organized, complex, formed images of small children playing in the snow. These visual phenomena occurred only in the occluded eye, began several hours after surgery, and recurred intermittently several times daily for 4 days, lasting several minutes with each occurrence.

Giant Ocular Horn Occurring in a 10-Year-Old Female.

Cutaneous horns uncommonly involve the periocular region. Involvement of the ocular surface is particularly rare. The authors present a patient who underwent a perinatal buccal mucosal graft for corneal perforation due to congenital corneal ectasia, most likely resulting from Peters anomaly.

Solitary Extramedullary Plasmacytoma of the Orbital Apex and Dura Masquerading as Idiopathic Inflammation.

This is a retrospective case description of a single female patient found to have a primary solitary extramedullary plasmacytoma of the orbital apex and temporal/parietal dura. These monoclonal plasma cell neoplasms account for fewer than 5% of plasma cell neoplasms. Although most commonly found in the head and neck, orbital and dural solitary extramedullary plasmacytomas are extremely uncommon.

Prolyl hydroxylase inhibition corrects functional iron deficiency and inflammation-induced anaemia in rats.

Background And Purpose: Small-molecule inhibitors of prolyl hydroxylase (PHD) enzymes are a novel target for the treatment of anaemia and functional iron deficiency (FID). Other than being orally bioavailable, the differentiation of PHD inhibitors from recombinant human erythropoietin (rhEPO) has not been demonstrated.

Experimental Approach: JNJ-42905343 was identified and characterized as a novel inhibitor of PHD and its action was compared with rhEPO in healthy rats and in a rat model of inflammation-induced anaemia and FID [peptidoglycan-polysaccharide (PGPS) model].

Physiological Control of Nitric Oxide in Neuronal BACE1 Translation by Heme-Regulated eIF2α Kinase HRI Induces Synaptogenesis.

Aims: Hippocampus is the brain center for memory formation, a process that requires synaptogenesis. However, hippocampus is dramatically compromised in Alzheimer's disease due to the accumulation of amyloid β-peptide, whose production is initiated by β-site APP Cleaving Enzyme 1 (BACE1). It is known that pathological stressors activate BACE1 translation through the phosphorylation of the eukaryotic initiation factor-2α (eIF2α) by GCN2, PERK, or PKR kinases, leading to amyloidogenesis.

Unilateral Prostaglandin-Associated Periorbitopathy: A Syndrome Involving Upper Eyelid Retraction Distinguishable From the Aging Sunken Eyelid.

Purpose: To study the effects of prostaglandin analogue drops on the eyelids and adnexa in unilaterally treated subjects with the intention of qualifying, quantifying, and categorizing the characteristics of prostaglandin-associated periorbitopathy (PAP).

Methods: Patients using prostaglandin analogue drops in only 1 eye for at least 1 year were evaluated by masked examiners. Orbital and eyelid measurements were obtained for each patient, and adnexal photographs were taken.