Retrobulbar Tissue Research Articles

Comparison between Widefield Optical Coherence Tomography Devices in Eyes with High Myopia.

Background: To compare four different optical coherence tomography (OCT) devices for visualization of retinal and subretinal layers in highly myopic eyes. Methods: In this prospective, observational, cross-sectional study, consecutive patients with high myopia and control subjects were imaged by four OCT devices: Spectralis OCT2, PlexElite 2.0 100 kHz, PlexElite 2.0 200 kHz and the Canon Xephilio OCT-S1. The acquisition protocol for comparison consisted of single vertical and horizontal line scans centered on the fovea. Comparison between the devices in the extent of visible retina, presence of conjugate image or mirror artifacts, visibility of the sclerochoroidal interface and retrobulbar tissue. Results: 30 eyes with high myopia and 30 control subjects were analyzed. The visualized RPE length was significantly different between the OCT devices with Xephilio OCT-S1 imaging the largest extent (p < 0.0001). The proportion of eyes with conjugate image artifact was significantly higher with the Spectralis OCT (p < 0.0001), and lower with the PlexElite 200 kHz (p < 0.0001). No difference in visibility of the sclerochoroidal interface was noted among instruments. The retrobulbar tissue was visible in a higher proportion of eyes using swept-source PlexElite 100 kHz and 200 kHz (p < 0.007) compared to the other devices. Conclusions: In highly myopic eyes, the four OCT devices demonstrated significant differences in the extent of the retina imaged, in the prevalence of conjugate image artifact, and in the visualization of the retrobulbar tissue.

A Promising Mouse Model of Graves' Orbitopathy Induced by Adenovirus Expressing Thyrotropin Receptor A Subunit.

Background: Graves' orbitopathy (GO) is the most common and serious manifestation of Graves' disease (GD). It is characterized by orbital inflammation and tissue remodeling. Although several GO models have been reported, most lack a full assessment or mechanistic evaluation. Here, we established a promising mouse model mimicking many aspects of human GO with a frequency of 70% and characterized the key role of T cells in the progression of GO. Methods: An adenovirus expressing the human thyrotropin (TSH) receptor A subunit (Ad-TSHRA) was injected in the muscles of female BALB/C mice nine times to induce GO. At predetermined time points, histological examinations of retrobulbar tissues and thyroid glands were performed to dynamically monitor changes; serum autoantibodies and total thyroxine levels were examined to evaluate thyroid function. Flow cytometry of CD4+ T cell subgroups and RNA sequencing (RNA-Seq) of splenocytes were also performed to explore the underlying mechanism. Results: After nine injections, 7 of 10 mice challenged with Ad-TSHRA developed the orbital changes associated with GO. Seven mice manifested retrobulbar fibrosis, and four mice showed adipogenesis. Exophthalmia, conjunctival redness, and orbital lymphocyte infiltration were also observed in a subset of mice. The orbitopathy was first detected after seven injections and followed the hyperplastic change observed in thyroids after four injections. Flow cytometry revealed increased proportions of Th1 cells and decreased proportions of Th2 cells and regulatory T (Treg) cells in the splenocytes of GO mice. This change in CD4+ T cell subgroups was confirmed by orbital immunohistochemical staining. Genes involved in T cell receptor signaling, proliferation, adhesion, inflammation, and cytotoxicity were upregulated in GO mice according to the RNA-Seq; a trend of upregulation of these GO-specific genes was observed in mice with hyperthyroidism without orbitopathy after four injections. Conclusions: A GO mouse model was successfully established by administering nine injections of Ad-TSHRA. The model was achieved with a frequency of 70% and revealed the importance of T cell immunity. A potential time window from Graves' hyperthyroidism to GO was presented for the first time. Therefore, this model could be used to study the pathogenesis and novel treatments for GO.

Prevalence of hyperthyroidism, hypothyroidism, and euthyroidism in thyroid eye disease: a systematic review of the literature

BackgroundThyroid eye disease is an autoimmune disorder of the orbital retrobulbar tissue commonly associated with dysthyroid status. The most frequent condition is hyperthyroidism, although it is also present in hypothyroid and euthyroid patients. The prevalence of thyroid conditions in patients with thyroid eye disease had been previously evaluated; however, there is no consensus on a global prevalence. The study aims to estimate the prevalence of hyperthyroidism, hypothyroidism, and euthyroidism in patients with TED, through a systematic review of literature.MethodsWe conducted a systematic review of the literature following the PRISMA guidelines, in MEDLINE, COCHRANE, EMBASE, Science Direct, and LILACS databases. Inclusion criteria were primary studies of patients with a diagnosis of thyroid eye disease made by an ophthalmologist or with diagnosis criteria, with measurement of thyroid function (TSH, T3, and free T4), and diagnosis of the primary thyroid condition. A quality assessment was made through the Joanna Briggs Institute Quality tools. Finally, we extracted relevant details about the design, the results, and the prevalence of thyroid disorders in thyroid eye disease.ResultsThe initial search revealed 916 studies, of which finally thirteen met inclusion criteria. Six studies were performed in Europe (Germany, Wales, and Spain), five in Asia (Iran, South Korea, Japan, and Singapore), one in North America (USA), and one in Africa (Ghana). The global prevalence, in patients of thyroid eye disease, was 10.36% for hypothyroidism, 7.9% for euthyroidism, and 86.2% for hyperthyroidism.ConclusionsProfessionals should be aware that thyroid eye disease can be present in patients with a normal thyroid function. The assessment for these patients is based on orbital images; serum TSH, T3, and free T4; antibody levels as thyrotropin receptor antibodies; and thyroperoxidase levels. Additionally, we want to encourage research in this field in other regions of the world such as Latin America.Systematic review registrationPROSPERO ID CRD42020107167

Expression of Collagen (Types I, III, and V), HSP47, MMP-2, and TIMP-1 in Retrobulbar Adipose Tissue of Patients with Thyroid-Associated Orbitopathy.

Objective This study aimed to investigate the expression of collagen (types I, III, and V), heat shock protein 47 (HSP47), matrix metalloproteinase-2 (MMP-2), and tissue inhibitors of metalloproteinase-1 (TIMP-1) in the retrobulbar adipose tissues of patients with thyroid-associated orbitopathy (TAO). Materials and Methods The retrobulbar adipose tissues were collected from 4 TAO patients undergoing orbital decompression and 4 ocular enucleation patients with atrophic eyeball caused by ocular trauma between May 2019 and September 2019. Masson staining was performed to analyze the differences in collagen expression and degree of histologic fibrosis in each sample. The protein expressions of collagen (types I, III, and V), HSP47, MMP-2, and TIMP-1 were determined by western blotting. The data of western blotting were analyzed using SPSS version 17.0, with independent t-tests. Results The results of Masson staining showed that the expression of collagen fibers in the TAO group was significantly higher than that in the control group, and the fibers were diffuse and irregular in distribution. The expression level of collagen (types I, III, and V), HSP47, MMP-2, and TIMP-1 in the TAO group were significantly higher than that in the control group (P < 0.05). Conclusion The proliferation and fibrosis of retrobulbar adipose tissue in TAO patients might be related to the increased expression of collagen (types I, III, and V) and HSP47 and decreased degradation of extracellular matrix.

Search of reference biomarkers reflecting orbital tissue remodeling in the course of Graves' orbitopathy.

Graves' orbitopathy (GO) is a complication in Graves' disease (GD) that causes disfigurement and sometimes blindness. The pathogenesis of GO remains unknown, while its symptoms demonstrate dependence between the thyroid gland and the orbit. The ongoing inflammatory process in retrobulbar tissue results in its remodeling characterized by increased volume of the orbital contents involving adipose tissue, with fibrosis and adipogenesis as predominant features. This study was aimed at the immunohistochemical verification of potential contribution and correlation between orbital expressions of IGF-1R, CD34, Foxp-3, PPAR-γ and CD4, CD68, TGF-β, FGF-β in severe and mild (long-lasting) GO. Forty-one orbital tissue specimens - 22 patients with severe GO, 9 patients with mild GO and 10 patients undergoing blepharoplasty as a control group - were processed by routine immunohistochemistry. Increased IGF-1R, CD34 and Foxp-3 expression was found in both severe and mild GO, yet a significant correlation between CD34 and CD4, CD68, TGF-β, FGF-β expressions was observed in long-lasting GO. CD34 expression is proposed to be the marker of orbital tissue remodeling in the course of mild GO.

Advanced Ocular Lesions in Growing Calves with Avitaminosis A

A beef cattle farm was visited due to blindness in a group of 28 calves aged 10-12 months. According to farmer, the first blind calves were observed about 20 days before and no sign of lacrimation or corneal opacity was observed. He first noticed that 3 calves were disoriented, hitting on the pen walls and preferred to stand near the feed and water troughs. Gradually, more than half of calves of this group developed similar signs. The calves were fed ad libitum with wheat straw and a homemade concentrate mixture consisting of corn, wheat bran, soybean meal and a mixture of vitamins and trace minerals. The mixture was added at the concentrate feed at the 1/10 of the recommended dosage. At animals’ inspection it was revealed that 18 out of 28 had the ocular lesions presented in (Figures 1 and 2). They were characterized by bilateral exophthalmos with dilated pupils. The degree of exophthalmos varied among animals. The pupils were unresponsive even in bright sunlight and the menace response was absent. The observed ocular lesions are typical of a vitaminosis A in young calves [1-3]. Blindness and absence of the pupillary light reflex are the result of optic nerve necrosis caused by stenosis of the optic foramen due to failure of bone resorption and increase inosteoblastic activity [1-4]. Exophthalmos is always bilateral, with the most advanced cases showing a prominent eye with large area of sclera visible all around cornea. It is regarded that protruding eyes are the result of blindness alone since no macroscopic lesions are observed at retro bulbar tissues [3]. Such advanced lesions are not reversible [1-3]; so, it was recommended all the affected animals to be slaughtered. It was suggested the rest of the animals to be treated with injectable administration of vitamin A along with the inclusion of the mixture of vitamins and trace minerals at the appropriate dosage in the concentrates.

Primer Açık Açılı Glokomlu Hastalarda Göz Elastisitesi

Purpose: The present study was performed to assess elasticity changes in the ocular and periocular structures of patients with primary open angle glaucoma POAG via sonoelastography. Patients and Methods: Forty-three patients 19 male, 24 female; ranging from 45 to 80, mean age, 63.20 ± 2.31 years diagnosed with POAG were prospectively analyzed. This patient group was compared with a control group of 28 individuals 17 male, 11 female; ranging from 40 to 85, mean age, 63.50 ± 1.89 years who had no eye diseases apart from refractive errors. Following an ophthalmologic assessment and B-mode sonography, all participants underwent semiquantitative sonoelastographic analysis. The elasticity of the retrobulbar fat tissue, optic nerve, lens, and retina–choroid–sclera complex RCSC was compared between the two groups via sonoelastography. Student’s t test and the Mann–Whitney U test were used for statistical assessment. Results: There was no significant differences in the degree of elasticity of the ocular and periocular structures between patients with and without POAG. Conclusion: No difference was detected between elasticity levels of ocular and periocular structures in between patients with and without POAG.

Radiotherapy for the treatment of primary intraocular lymphoma and orbital (ocular adnexa) lymphomas

AbstractPrimary intraocular lymphoma (PIOL) is a lymphomatous proliferation seen in the eye and can be subdivided into two groups: (1) those which occur in the vitreous and /or retina and (2) those which occur in the uvea [1]. The histology is usually diffuse large B‐cell lymphoma (DLBCL) and diagnosis is made by vitrectomy, vitreal biopsy, or choroidal sampling. Vitreoretinal lymphomas are lymphomas that arise primarily in the vitreous and/or the retina and are considered as a part of the primary central nervous system lymphomas (PCNSL). Primary uveal lymphomas start as a primary disease in the uveal tract. Because brain was the most common site of relapse, some old data recommended in the past to treat as for PCNSL [2]. Modern data suggest that in case of brain relapse, salvage tratements remain effective [3]. Most patients with PIOL present a high risk of bilateral involvement, even when pathologic results have been obtained from only one eye. Ocular irradiation should include both eyes. However, when there is no suspicion of disease in the contralateral eeye, it is reasonable to treat onlym the involved eye. The volume of irradiation has to include the globe of the eye (s), optic nerves (s) to the level of the chiasm. Low dose of 30‐36 Gy is recommended [4]. Primary lymphoma of the ocular adnexa (orbital lymphoma) is a very rare tumor entity and accounts less than 1% of all non‐Hodgkin lymphomas (NHL) and 8% of all extranodal NHL [5]. Most cases of extraocular orbital lymphoma are marginal zone lymphoma (MZL) and involve the conjunctiva, lacrimal gland, eyelid, or retrobulbar soft tissues. Less common are follicular lymphoma (FL) and DLBCL. Primary radiation therapy (RT) is the curative option in patients with indolent lymphoma with high response rate and acceptable toxicity [6]. RT may be indicated to consolidate systemic therapy for DLBCL. For most cases of indolent NHL, the entire bony orbit has to be included. When the disease is limited to the conjunctiva, the irradiation has to include the entire conjunctival sac and local extensions to eyelid. A standard dose of 24 to 25 Gy in 1.5 to 2 Gy per fraction is prescribed. Since indolent lymphomas are highly radiosensitive, low dose irradiation (4 Gy in 2 fractions of 2 Gy) has been used in order to minimize the toxicity. A randomized non‐inferiority trial (FORT) compared conventional radiation scheme (24 Gy) to low dose RT (4Gy) in patients with FL. Low dose RT showed inferior results in term of local progression free survival [7].REFERENCES Venkatesh R, Bavaharan B, Mahendradas P, Yadav NK. Primary vitreoretinal lymphoma: prevalence, impact, and management challenges. Clin Ophthalmol. 2019;13:353‐64. Akpek EK, Ahmed I, Hochberg FH, Soheilian M, Dryja TP, Jakobiec FA, et al. Intraocular‐central nervous system lymphoma: clinical features, diagnosis, and outcomes. Ophthalmology. 1999;106:1805‐10. Teckie S, Yahalom J. Primary intraocular lymphoma: treatment outcomes with ocular radiation therapy alone. Leuk Lymphoma. 2014;55:795‐801. Yahalom J, Illidge T, Specht L, Hoppe RT, Li YX, Tsang R, et al. Modern radiation therapy for extranodal lymphomas: field and dose guidelines from the International Lymphoma Radiation Oncology Group. Int J Radiat Oncol Biol Phys. 2015;92:11‐31. Margo CE, Mulla ZD. Malignant tumors of the orbit. Analysis of the Florida Cancer Registry. Ophthalmology. 1998;105:185‐90. Fakhrian K, Klemm S, Keller U, Bayer C, Riedl W, Molls M, et al. Radiotherapy in stage I‐III follicular non‐Hodgkin lymphoma. Retrospective analysis of a series of 50 patients. Strahlenther Onkol. 2012;188:464‐70. Hoskin PJ, Kirkwood AA, Popova B, Smith P, Robinson M, Gallop‐Evans E, et al. 4 Gy versus 24 Gy radiotherapy for patients with indolent lymphoma (FORT): a randomised phase 3 non‐inferiority trial. Lancet Oncol. 2014;15:457‐63.

The role of tissue hypoxia and endothelial activation in the pathogenesis of graves’ ophthalmopathy

The study included 47 people (18 men and 29 women) at the age of 43 [35; 50] years. A clinical group was formed by 32 patients with moderate severity of GO. The control group consisted of 15 healthy individuals. Opthalmologic examination and blood sampling were conducted to determine the concentration of antibodies to the thyroid stimulating hormone receptor (TSAbs), hypoxia-inducible factor-1α (HIF-1α) and soluble vascular cell adhesion molecule-1 (sVCAM-1). An increase in the sVCAM-1 concentration was revealed in all patient of the clinical groups in comparison with the control (p&lt;0,05). There were no statistically signifi cant diff erences in HIF-1α level in all groups (p&gt;0,05). A direct correlation between sVCAM-1 and HIF-1α concentration was found (r=0,343; p&lt;0,05), which probably indicates that factors contributing to the development of tissue hypoxia can activate endothelial cells and may intensify the infl ammatory processes of soft retrobulbar tissues.

Matrix metalloproteinases-1, -13 and their tissue inhibitor-1 in endocrine ophthalmopathy

The study included 65 people (130 eyes) at the age of 43 (35-50) years. Three groups of subjects were formed: 32 patients with a moderate GO severity (clinical group), 18 patients with autoimmune thyroid pathology without GO (comparison group), and 15 healthy persons (control). The diagnosis was based on clinical, laboratory, and instrumental data. A comprehensive ophthalmologic examination and blood sampling for determination of MMP-1, -13, TIMP-1, sulfated glycosaminoglycans (sGAG) and antibodies to thyroid-stimulating hormone receptor (TSHRAbs) were conducted. The data were statistically processed using the program Statistica 10.0. An elevated level of MMP-13, observed in all GO patients (p<0.05). For the active phase of GOP, the comparison with the control group showed a 3.5-fold increase in MMP-13 (p<0.001) and 1.17-fold rise in TIMP-1 (p>0.05). Pulse glucocorticoid therapy reduced MMP-13 by 48.6% (p<0.001), TIMP-1 by 2.7% (p<0.001), and TSHRAbs - by 93% (p<0.001) compared with active GO, but these indicators were higher than the reference limits of control (p>0.05). In inactive GO, despite increased MMP-13, TIMP-1 decreased to the reference values (p=0.533). There were no significant differences in MMP-1 in groups of subjects (p=0.865). We have found imbalance between MMP-13 and TIMP-1 production in different activity phases of GO. Active GO is characterized by an increase in serum MMP-13 and TIMP-1. Dysregulation of intercellular matrix remodeling, possibly, underlies the development of extraocular muscles and retrobulbar tissue fibrosis in GO.

Morphofunctional characteristics and immunological regulation of the orbital fibroblasts function in endocrine ophthalmopathy

Endocrine ophthalmopathy (EOP) is a chronic disease characterized by progressive autoimmune inflammation of the soft retrobulbar tissues in thyroid dysfunction. The orbital fibroblasts with their unique morphofunctional properties are very important in the pathogenesis of the infiltrative process and fibrosis of the extraocular muscles and/or retrobulbar tissue. They, unlike other localization fibroblasts, have not mesodermal, but neuro-ectodermal origin. The review acquaints with the immunological aspects of the regulation of these cells in different activity phases of disease. Intercellular interaction with T-lymphocytes (CD40-CD154) leads to orbital fibroblasts activation with increased expression of pathological receptors for thyroid-stimulating hormone, as well as production of intercellular matrix components, adhesion molecules, growth factors, cytokines and prostaglandins. Detailed morphofunctional characteristics of the orbit fibroblast subpopulations and mechanisms regulating their transdifferentiation into adipocytes and myofibroblasts are given. The analysis of literature data on the effect of T-helper type 17 on the functional activity of Thy1+/Thy1- (CD90+/CD90-) orbital fibroblasts is presented. The importance of the further study of the orbital fibroblasts characteristics in EOP and their intercellular interaction with various immune cells was noted, which may be able to uncover new pathogenetic mechanisms of this pathology.

The features of cataract surgery in a patient with Graves’ disease and endocrine ophthalmopathy

After the phacoemulsification technique was implemented in ophthalmic practice surgeries to replace the clouded lens have become routine for ophthalmologists. Today, over half a million phacoemulsification surgeries are annually conducted in Russia. Cataract combined with endocrine ophthalmopathy poses a significant challenge for an operating surgeon because of the unusual anatomical presentation of the orbit and the eyeball, as well as the elevated intraocular pressure caused by edema of the orbital tissues (i.e., retrobulbar tissue and extraocular muscles) rather than by primary glaucoma.&#x0D; We analyze the features of surgical treatment of the cataract involving intraocular lens implantation in a patient with Graves’ disease and endocrine ophthalmopathy complicated by secondary ocular hypertension, optical neuropathy, and lagophthalmos.&#x0D; After restoring the euthyroid state and reducing the severity of endocrine ophthalmopathy, phacoemulsification was performed in both eyes, with an interval of 2 months. Neither intra- nor postoperative complications of the patients’ organ of vision were observed. The maximum corrected visual acuity achieved on both eyes was 0.4–0.5 in the Snellen eye chart.

Demystifying Role of Ultrasound Elastography in the Evaluation of Primary Open Angle Glaucoma

For this cross-sectional comparative study, 20 patients with primary open-angle glaucoma (POAG) and 20 healthy volunteers were recruited. Ultrasound elastography measurements were taken with a sonographic scanner of the RCS complex, anterior vitreous (AV), posterior vitreous (PV), retrobulbar fat tissue (RFT), optic disc, and optic nerve in each eye.

The role of interleukins 17, 23 and antibodies to the thyroid-stimulating hormone receptor in the pathogenesis of endocrine ophthalmopathy

Background. Endocrine ophthalmopathy (EOP) is an autoimmune orbit disease characterized by soft retrobulbar tissues damage. The level of antibodies to the thyroid-stimulating hormone receptor (TSHRAbs) is considered as a laboratory marker of EOP activity. Interleukins 17 (IL-17) and 23 (IL-23) play an important role in the pathogenesis of some autoimmune diseases and directly correlate with clinical activity. At present, there is an open question about the role of these cytokines in EOP and their relationship with TSHRAbs.&#x0D; Aims. To assess pathogenetic role of IL-17, IL-23 and TSHRAbs in patients with EOP.&#x0D; Materials and methods. The study included 50 people (100 eyes) at the age of 43 [35; 50] years. Three study groups were formed: 32 patients with moderate severity of EOP (clinical group), 18 patients with thyroid pathology without EOP (comparison group) and 15 healthy subjects (control group). All groups were comparable in age and sex. The diagnosis was verified clinically, laboratory and instrumentally. A comprehensive ophthalmologic examination and blood sampling were performed to determine the concentrations of IL-17, IL-23 and TSHRAbs. Statistical processing of the data was carried out in the program “Statistica 10.0”, StatSoft, Inc.&#x0D; Results. An increase in the level of TSHRAbs was observed in all phases of EOP activity in comparison with both comparison group and control (p &lt; 0.05). But in the active phase TSHRAbs level reached the maximum values in 100% of patients. An increase in the IL-17 concentration in 5,3 times was found in the active EOP in comparison with the control group (p &lt; 0.05). Concentration of TSHRAbs and IL-17 in blood serum directly correlates with EOP activity (p &lt; 0.001). After carrying out pulse therapy with glucocorticosteroids, the consentration of IL-17 decreased almost to zero. There were no significant differences in the level of IL-23 in the groups (p = 0.56).&#x0D; Conclusions. Determination of TSHRAbs and IL-17 levels in serum can be used as a laboratory diagnostic marker of EOP activity.&#x0D;

Varicella zoster virus reactivation antedating ipsilateral brainstem stroke

itor Title: Varicella zoster virus reactivation antedating ipsilateral brainstem stroke Authors: Giuliana Galassi1, Maurilio Genovese2, Marisa Meacci3, Marcella Malagoli2 Affiliations: 1Department of Biomedical, Metabolic, Neural Sciences, University Hospital of Modena, Italy, 2Neuroradiology Service, University Hospital of Modena, Italy, 3Department of Laboratory Medicine and Patholgy, Microbiology and Virology Unit, University Hospital of Modena, Italy Corresponding Author: Giuliana Galassi, MD, Department of Biomedical, Metabolic, Neural Sciences, University Hospital of Modena, Via P. Giardini 1455, Modena, Italy, Tel: 39-3497325802, Email: giulianagalassi46@gmail.com Abstract: Varicella zoster virus (VZV) infection and reactivation are associated with a number of neurologic conditions. Unifocal large vessel infarcts may follow zoster in the trigeminal or cervical distribution as a result of transaxonal transport of virus from trigeminal or cervical afferent fibers that innervate vessels. Ophthalmic zoster (HZO) might cause ophthalmoplegic syndromes, with secondary optic neuritis. Mechanisms include local orbital muscle inflammation and, viral spread from the ophthalmic branch of the fifth nerve with associated vasculopathy. A 72-year-old man developed a vesicular rash in the territory of C5-T5-6. Within four weeks, the patient developed headache, dysphagia, left facial and extremity ataxic weakness. Magnetic resonance imaging (MRI) revealed a right pontine infarction. A 66-year-old woman presented with right-sided painfull HZO. One week later she developed complete external ophthalmoplegia and blurred vision. MRI showed ill-defined signal alteration in the retrobulbar tissue. Three weeks later, the patient was admitted because of dysarthria, deviated tongue, left-sided limb weakness, and tactile hypoesthesia. Spinal fluid contained 23 lymphocytes/mm3 and increased protein. The serum contained antibodies to VZV IgG and IgM in both cases. The patients received intravenously acyclovir with improvement. This report confirms unusual occurrence of ipsilateral brainstem stroke after VZV reactivation in immunocompetent subjects.

Diagnosis of IgG4 - related ophthalmic disease in a group of patients with various lesions of the eye and orbits.

To provide demographic, clinical, laboratory, ultrasound, radiological, morphological/ immunomorphological phenotype of IgG4-related ophthalmic diseases, which allowsmaking a differential diagnosis with granulomatous, autoimmune, inflammatory, endocrine and hematologic diseases affecting the eye and orbits. From 2004 to 2016 108 (78.2%) of the 138 patients were diagnosed with non-tumoral lesions of eye and orbits. In 48 patients (35%) at admission and 5 patients in the follow were diagnosed IgG4-related ophthalmic disease. In the analysis of 82 (f-44, m-38) patients with IgG4-related disease, localization of lesions in orbit observed in 53 (f-36, m-17) and it was the most frequent involvement in patients with IgG4-related disease (64.5%). Only 7 patients had isolated IgG4-related ophthalmic disease, whereas 46 patients (87%) had involvement of 2-7 locations, as a manifestation of IgG4-related systemic disease.During the examination, the average age of patients with IgG4-related ophthalmic disease was 47.5 years (19-73 years). Median time to diagnosis was 52.8 months before 2004 and 36 months 2004-2016. We noted the predominance of females in the ratio 2: 1 inthe group of patients with IgG4-related ophthalmic disease. Edema of the eyelids, nasal congestion (55-60%), tumor-like formations of the upper eyelids and increased lacrimation prevailed at the onset of the disease, whereas such functional impairment like limited mobility and pain in eyeballs, exophthalmos, ptosis and diplopia appeared later at 15-38% with a loss visual acuity in one case. Bilateral lesion (86%), mainly affecting the lacrimal glands (93.5%), infiltration of the ex- traocular muscles (83.5%) and retrobulbar tissue with a thickening of the optic nerve in one third of patients were the main localizations IgG4-related ophthalmic disease. Clinical symptoms were accompanied by the appearance of moderate inflammatory activity (38%), in- creased levels IgG (44%), IgG4(88%) and IgE (61%). Indicators of autoimmune disorders observed in 6-22% of patients, most often in pa- tients with simultaneous involvement of the salivary glands. Significant lymphoplasmacytic infiltration (94%) with a ratio of plasma cells (IgG4/IgG) secreting IgG4&gt; 40% (90%) with fibrosis formation (94%) and follicle formation (71%) with a moderate amount of eosinophils (34%) were the major morphological / immunomorphological manifestations of IgG4-related ophthalmic disease. Signs of vasculitis and obliterative phlebitis were found in a small amount of patients. Determination of elevated levels of IgG-4 / IgE in patients with edema, pseudotumor of the eyelid, sinusitis and increase of the palpebral lobe of the lacrimal gland suggests the presence of IgG4-related ophthalmic disease. Minimally invasive incisional biopsy of lacrimal glands and salivary glands followed by morphological / immunomorphological research is needed for the correct diagnosis. Diagnostic orbitotomy in ophthalmic hospitals in such cases is inexpedient, since it leads to the development of dry eye. Massive lymphoplasmacytic infiltration with IgG4 / IgG ratio more than 40%, advanced fibrosis in biopsiesof the orbits tissue or salivary glands when combined lesions are required for the making the diagnosis of IgG4-related ophthalmic disease.

New opportunities in endocrine ophthalmopathy diagnostics (review)

Эндокринная офтальмопатия (ЭОП) – это прогрессирующее аутоиммунное заболевание, поражающее ретробульбарные мягкие ткани на фоне заболеваний щитовидной железы. Представлена статистическая информация по данному заболеванию. В обзоре рассматриваются как общепринятые, так и альтернативные подходы к диагностике разных стадий ЭОП. Дана подробная клиническая симптоматика, основные классификации определения степени тяжести и активности ЭОП, необходимый перечень физикальных и инструментальных обследований. Описана диагностическая ценность компьютерной томографии, магнитно-резонансной томографии, оптической когерентной томографии, Гейдельбергской ретинальной томографии, радиоизотопных исследований, триплексного сканирования магистральных сосудов глаза, метода доплеровского картирования, индоцианин-зеленой ангиографии, а также гистологического исследования биоптатов мягких ретробульбарных тканей при ЭОП. Общепринятые методы диагностики ЭОП имеют свои недостатки. Поэтому в настоящее время перспективным направлением являются иммунологические, биохимические и генетические исследования маркеров активности ЭОП. Особенностью данных методов в диагностике ЭОП является точность, возможность многократного измерения, минимум побочных эффектов, а также относительно низкая стоимость. Отмечена важность дальнейшего изучения ключевых механизмов развития отека, лейкоцитарной инфильтрации с последующим формированием фиброза экстраокулярных мышц и ретробульбарной клетчатки при ЭОП, что, возможно, сможет улучшить диагностику в клинически сложных случаях и при начальных стадиях заболевания.

Orbital decompression : Indications, technique, results

Orbital decompression is an effective surgical procedure to reduce intraorbital pressure. Causes may diseases leading to rapid pressure increases, e. g., bleedings, and those causing slower, progressive pressure increases, e. g., tumors or Graves' orbitopathy. During fat tissue decompression, peri- and retrobulbar adipose tissue is removed; in bony decompression, one or more bony orbital walls are removed (one-, two-, or three-wall decompression). In many cases the procedures are combined. Recent developments are the transconjunctival approaches for removing parts of bony orbital walls. Complications include double vision, which occurs in up to 30% of cases depending on the approach, hemorrhage, infections, development of chronic sinusitis, and iatrogenic skull base lesions with consecutive meningitis. In the hands of an experienced rhino- and head and neck surgeon, the intervention has low complication rates.

VOLUME MEASUREMENTS OF NORMAL BONY ORBIT AND ORBITAL SOFT TISSUE IN CHILDREN BY COMPUTED TOMOGRAPHIC ANALYSIS

Objective: to study the volumetric data of the bone of the orbit and its soft tissues in children is normal. Material and methods. A research into CT scans of 50 children (80 orbits) without orbital abnormality was conducted. The computed tomography imaging was performed routinely, with both axial and frontal sections obtained. Section thickness measured 1.0 mm at intervals of 1.00 mm. Results. The article presents volume measurements for normal bony orbits and orbital soft tissue (extraocular muscle, retrobulbar tissue, optic nerve, and lacrimal gland) in children in vivo, with regard to age peculiarities. The orbital tissue volume ratios presented in the article allow for conclusions about age-specific changes in the anatomy and topography of the orbit in children. Conclusion. The growth of a child is accompanied by a continuous steady increase in the bony orbit and orbital soft tissue volume, which reaches 21.93 cm3 and 14.87 cm3 respectively by the age of 15 to 16 years. With age, the volume of orbital soft tissues shows a greater increase than the volume of the bony orbit, which leads to a change in topographic ratio of orbital tissues (the muscle “cone” widens, the eyeball protrudes forward).

An unusual Erdheim-Chester disease with orbital involvement: a case report

Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis with multiorgan involvement and a specific tropism for perivascular and fatty connective tissue, of unclear origin, with poor response to therapy. Its identification is difficult because of the variable clinical presentation and its lack of knowledge. We report the case of a 63-years-old woman, with a history of bilateral orbital pseudotumor, who comes to our attention because of progressively worsening asthenia, vomiting and systemic inflammation. Total body computerized tomography scan showed a volumetric increase of choroid plexus of the temporal horn of the left lateral ventricle, presence of solid retrobulbar tissue at the level of both maxillary sinuses, lung fibrosis, and retroperitoneal and peri-aortic infiltration. The association of these signs addressed to a diagnosis of Erdheim- Chester disease. Thus, although extremely rare, the diagnosis of Erdheim-Chester disease must be considered in the case of bilateral retro-orbital tumors and multisystemic involvement.