Primary ductal adenocarcinoma of the lacrimal gland: A review and report of five cases.

Primary ductal adenocarcinoma (PDA) is a rare epithelial tumor of the lacrimal gland. Herein we report 5 cases and review 29 published cases of PDA of the lacrimal gland. Among these 5 cases, the most common clinical presentation was painless swelling and/or proptosis of their eye. The size of the lesions ranged from 1.6 to 2.5 cm. Histopathologic examination revealed proliferations of ductal or gland-like cells with vesiculated pleomorphic nuclei and prominent nucleoli. Tumor cells stained positive for epithelial and apocrine differentiation markers. Immunohistochemistry for human epidermal growth factor 2 was positive in 2 of the 4 cases. Four of the five patients were alive at the last follow-up visit. One died with bone metastases, which were diagnosed 25 months after exenteration and then survived an additional 51 months. On reviewing of twenty-nine previously published cases of PDA, the mean age of diagnosis was 58 years, with a male predominance (75%). Fifteen patients (54%) had distant metastases, 1 (4%) had local recurrence, and 10 (37%) suffered from a PDA-related death. PDA is a high-grade aggressive epithelial tumor of the lacrimal gland. Although rare, awareness and recognition of this malignancy are important to help determine prognosis and treatment options.

Diversity in the American Society of Ophthalmic Plastic and Reconstructive Surgery.

PURPOSE: To assess the epidemiology of women and underrepresented minorities (URMs; Hispanic, African American, and Native American) in the American Society of Ophthalmic Plastic and Reconstructive Surgery (ASOPRS). METHODS: An observational retrospective study of living ASOPRS members was identified through the ASOPRS member directory, search engine-driven informatics, and direct communication. Members were profiled for gender, race, geographic location, and academic rank (or not). The percentage of women and URMs in the society over time was also assessed. RESULTS: The authors identified 617 living ASOPRS members as of June 2017. Of these, 109 (17.7%) were female and 58 (9.4%) were URMs. Surgeons completed fellowships from 1961 (male) or 1973 (female) until 2015. Women members significantly increased from 7 (4.9% of the total) before 1986 to 24 (30.4%) between 2011 and 2015 (last 5 years). URMs significantly increased over time, with 0 female and 5 (3.4%) male minorities before 1985 to 5 (6.3%) females and 13 (16.5%) males within the last 5 years (p < 0.05). With only 1 female and 3 male members, African American representation was sparse. ASOPRS members were not found in 5 states and were most represented in California, New York, Florida, and Texas. Among ASOPRS members in full-time academic positions, women tended to have lower rank compared to men; however, after controlling for number of years post-fellowship, this finding was narrowly not statistically significant (p = 0.0624). There were no academic differences with URMs and nonminority groups. CONCLUSIONS: Women and URMs have increased steadily in ASOPRS, especially in recent years. Similar to the rest of ophthalmology and general medicine, there remain opportunities for ASOPRS to increase diversity.

Baby Shampoo Versus Povidone-Iodine or Isopropyl Alcohol in Reducing Eyelid Skin Bacterial Load.

PURPOSE: Baby shampoo is used as an alternative surgical skin preparation, but the evidence supporting its use is scarce with no descriptions of efficacy in the periocular region. The authors compare the efficacy of baby shampoo, povidone-iodine (PI, Betadine) and isopropyl alcohol (IA) in reducing eyelid skin bacterial load. METHODS: Prospective, randomized, comparative, and interventional trial. Bacterial load on adult, human eyelid skin was quantitated before and after cleansing with 1) dilute baby shampoo, 2) 10% PI, or 3) 70% IA. Paired skin swabs were collected from a 1 cm area of the upper eyelid of subjects before and after a standardized surgical scrub technique. Samples were cultured on 5% sheep blood agar for 24 hours. The number of colony forming units (CFU) was assessed and bacterial load per square centimeter of eyelid skin was quantified. RESULTS: Baseline and postcleansing samples were assessed from 42 eyelids of 42 subjects (n = 14 for each of baby shampoo, PI, and IA). Before cleansing, similar amounts of bacterial flora were grown from all specimens (median log CFU/cm = 2.04 before baby shampoo, 2.01 before PI, 2.11 before IA; p > 0.05). All 3 cleansing agents significantly reduced the bacterial load (p < 0.01 for each). There was no statistically significant difference in postcleansing bacterial load between the 3 cleansing agents (median log CFU/cm = 0.48 after baby shampoo, 0.39 after PI, 0.59 after IA; p > 0.05). Change from baseline in bacterial load was statistically similar for all 3 agents (median reduction in log CFU/cm = 1.28 with baby shampoo, 1.57 with PI, 1.40 with IA; p > 0.05). These corresponded to bacterial load reductions of 96.3%, 96.6%, and 98.4% for baby shampoo, PI, and IA, respectively. CONCLUSIONS: Baby shampoo achieved comparable diminution in eyelid skin bacterial load to PI or IA. These data suggest baby shampoo may be an effective preoperative cleansing agent.

A Case of Attempted Bilateral Self-Enucleation in a Patient with Bipolar Disorder.

Attempted and completed self-enucleation, or removal of one's own eyes, is a rare but devastating form of self-mutilation behavior. It is often associated with psychiatric disorders, particularly schizophrenia, substance induced psychosis, and bipolar disorder. We report a case of a patient with a history of bipolar disorder who gouged his eyes bilaterally as an attempt to self-enucleate himself. On presentation, the patient was manic with both psychotic features of hyperreligous delusions and command auditory hallucinations of God telling him to take his eyes out. On presentation, the patient had no light perception vision in both eyes and his exam displayed severe proptosis, extensive conjunctival lacerations, and visibly avulsed extraocular muscles on the right side. An emergency computed tomography scan of the orbits revealed small and irregular globes, air within the orbits, and intraocular hemorrhage. He was taken to the operating room for surgical repair of his injuries. Attempted and completed self-enucleation is most commonly associated with schizophrenia and substance induced psychosis, but can also present in patients with bipolar disorder. Other less commonly associated disorders include obsessive-compulsive disorder, depression, mental retardation, neurosyphilis, Lesch-Nyhan syndrome, and structural brain lesions.

Differentiating Urgent and Emergent Causes of Acute Red Eye for the Emergency Physician.

Patients commonly present with an acute red eye to the emergency department (ED). It is important to distinguish between benign and sight-threatening diagnoses. Here we provide a comprehensive overview on the acute red eye in the ED.

Orbital Fracture Repair.

Orbital fractures are very common after facial trauma. The assessment of a patient with a suspected orbital wall injury includes a detailed oculofacial examination as well as radiologic imaging. Surgical repair with or without an implant may be indicated for diplopia, enophthalmos, or both. Cicatricial eyelid malposition is an iatrogenic complication commonly due to poor orbitotomy technique. Optimal repair involves direct exposure of the perimeter of the fractures' site through surgical planes that minimally scar the eyelids. A wide variety of implant options exist; however, thin, pliable, nonadherent materials such as nylon foil may offer several advantages. The authors describe the evaluation and management of orbital wall fractures.

Vascular Steal Syndrome, Optic Neuropathy, and Foreign Body Granuloma Reaction to Onyx-18 Embolization for Congenital Orbito-Facial Vascular Malformation.

A 34-year-old patient presented with a right orbito-facial mass since childhood, consistent with a congenital arteriovenous (AV) malformation. Prior to presentation, she had multiple incomplete surgical resections and embolizations with N-butyl acetyl acrylate and Onyx-18. The patient reported gradual, progressive vision loss shortly after Onyx-18 embolization. Five months after embolization, she presented with decreased vision, disfigurement and mechanical ptosis relating to a large subcutaneous mass affecting the medial right upper eyelid and forehead. Significant exam findings included a visual acuity of 20/400 (20/60 prior to embolization), an afferent pupillary defect, and optic disc pallor. MRI and angiography revealed a persistent AV malformation with feeders from the ophthalmic artery and an absent choroidal flush to the right eye. Pathology from surgical resection showed a significant foreign body giant cell reaction to the embolization material adjacent to the vessels. We suggest that an incomplete embolization with Onyx-18 may have caused vascular steal syndrome from the ophthalmic artery.

Molecular components underlying nongenomic thyroid hormone signaling in embryonic zebrafish neurons.

BACKGROUND: Neurodevelopment requires thyroid hormone, yet the mechanisms and targets of thyroid hormone action during embryonic stages remain ill-defined. We previously showed that the thyroid hormone thyroxine (T4) rapidly increases voltage-gated sodium current in zebrafish Rohon-Beard cells (RBs), a primary sensory neuron subtype present during embryonic development. Here, we determined essential components of the rapid T4 signaling pathway by identifying the involved intracellular messengers, the targeted sodium channel isotype, and the spatial and temporal expression pattern of the nongenomic alphaVbeta3 integrin T4 receptor. RESULTS: We first tested which signaling pathways mediate T4's rapid modulation of sodium current (I(Na)) by perturbing specific pathways associated with nongenomic thyroid hormone signaling. We found that pharmacological blockade of protein phosphatase 1 and the mitogen-activated protein kinase p38 isoform decreased and increased tonic sodium current amplitudes, respectively, and blockade of either occluded rapid responses to acute T4 application. We next tested for the ion channel target of rapid T4 signaling via morpholino knock-down of specific sodium channel isotypes. We found that selective knock-down of the sodium channel alpha-subunit Na(v)1.6a, but not Na(v)1.1la, occluded T4's acute effects. We also determined the spatial and temporal distribution of a nongenomic T4 receptor, integrin alphaVbeta3. At 24 hours post fertilization (hpf), immunofluorescent assays showed no specific integrin alphaVbeta3 immunoreactivity in wild-type zebrafish embryos. However, by 48 hpf, embryos expressed integrin alphaVbeta3 in RBs and primary motoneurons. Consistent with this temporal expression, T4 modulated RB I(Na) at 48 but not 24 hpf. We next tested whether T4 rapidly modulated I(Na) of caudal primary motoneurons, which express the receptor (alphaVbeta3) and target (Na(v)1.6a) of rapid T4 signaling. In response to T4, caudal primary motoneurons rapidly increased sodium current peak amplitude 1.3-fold. CONCLUSION: T4's nongenomic regulation of sodium current occurs in different neuronal subtypes, requires the activity of specific phosphorylation pathways, and requires both integrin alphaVbeta3 and Na(v)1.6a. Our in vivo analyses identify molecules required for T4's rapid regulation of voltage-gated sodium current.

Sensory neuron sodium current requires nongenomic actions of thyroid hormone during development.

Development of the embryonic nervous system requires thyroid hormone. However, the underlying mechanisms and targets of thyroid hormone action are not well defined. To identify embryonic roles for thyroid hormone we tested for effects on a key neuronal trait, voltage-gated sodium current (I(Na)), in the zebrafish model system. We recorded from Rohon-Beard sensory neurons (RBs) using whole cell voltage-clamp methods. Here, we provide in vivo evidence for thyroid hormone regulation of I(Na). Chronic thyroid hormone application increased RB peak I(Na) density 1.4-fold. However, I(Na) density showed a similar increase within 5 min of an acute hormone application, a time course not expected for a genomic mechanism. Tetraiodothyroacetic acid (tetrac), a thyroid hormone blocker, blocked both chronic and acute effects. Further, the thyroid hormone precursor thyroxine (T4) affected I(Na), yet the traditionally active form triiodothyronine did not. Consequently, we tested for a nonconventional T4 receptor. LM609, a selective antagonist of integrin alphaVbeta3, occluded the rapid effect of T4, implicating a specific integrin dimer as a T4 receptor. Chronic application of either tetrac or LM609 significantly reduced sodium conductance, demonstrating an in vivo requirement for T4-integrin regulation of I(Na). Further, removing endogenous T4 levels via yolkectomy reduced sodium conductance, an effect that was partially rescued by T4 supplementation following surgery. Because RBs mediate the embryonic touch response, we tested for behavioral effects. Tetrac and LM609 significantly reduced the percentage of touch trials eliciting a normal touch response. T4's rapid effect on RB I(Na) highlights the importance of embryonic T4 availability and nongenomic T4 signaling.

Contribution of persistent sodium currents to spike-frequency adaptation in rat hypoglossal motoneurons.

In response to constant current inputs, the firing rates of motoneurons typically show a continuous decline over time. The biophysical mechanisms underlying this process, called spike-frequency adaptation, are not well understood. Spike-frequency adaptation normally exhibits a rapid initial phase, followed by a slow, later phase that continues throughout the duration of firing. One possible mechanism mediating the later phase might be a reduction in the persistent sodium current (I(NaP)) that has been shown to diminish the capacity of cortical pyramidal neurons and spinal motoneurons to sustain repetitive firing. In this study, we used the anticonvulsant phenytoin to reduce the I(NaP) of juvenile rat hypoglossal motoneurons recorded in brain stem slices, and we examined the consequences of a reduction in I(NaP) on the magnitude and time course of spike-frequency adaptation. Adding phenytoin to the bathing solution (> or =50 microM) generally produced a marked reduction in the persistent inward currents (PICs) recorded at the soma in response to slow, voltage-clamp triangular ramp commands (-70 to 0 mV and back). However, the same concentrations of phenytoin appeared to have no significant effect on spike-frequency adaptation even though the phenytoin often augmented the reduction in action potential amplitude that occurs during repetitive firing. The surprising finding that the reduction of a source of sustained inward current had no appreciable effect on the pattern of spike generation suggests that several types of membrane channels must act cooperatively to insure that these motoneurons can generate the sustained repetitive firing required for long-lasting motor behaviors.