[Transnasal endoscopic orbital decompression in the algorithm of surgical treatment of endocrine ophthalmopathy]. / Transnazal'naya endoskopicheskaya dekompressiya orbity v algoritme khirurgicheskogo lecheniya endokrinnoi oftal'mopatii.

OBJECTIVE: To develop an algorithm for the use of transnasal endoscopic orbital decompression (TEOD) in endocrine ophthalmopathy (EOP). MATERIAL AND METHODS: The results of 225 TEODs are analyzed. 139 patients with EOP were under observation, who were divided into two groups. Group 1 included 64 patients with an inactive form of EOP, group 2 included 75 patients with EOP complicated by optical neuropathy. Group 2 patients are divided into three subgroups. In the first subgroup there were 36 patients who underwent only TEDO, in the second subgroup there were 17 patients who underwent simultaneous TEOD and lateral bone orbital decompression (LBOD), in the third subgroup there were 22 patients who underwent TEOD and with an interval of 7-14 days - LBOD. After surgery, visual acuity, visual fields and color perception, regression of exophthalmos, as well as the presence of strabismus and diplopia were evaluated. RESULTS: In patients of group 1 (64 patients, 104 TEODs), the average regression rate of exophthalmos was 5.49±1.22. Diplopia was observed in 23 (36%) patients, strabismus - in 18 (28%) patients. The transient nature of diplopia and strabismus was noted in 11% of cases. Patients of the first subgroup showed an increase in visual acuity from 0.4±0.28 to 0.7±0.2 (p<0.05). Strabismus and diplopia were observed in 54% of cases. In the subgroup with step-by-step performed TEOD and LBOD, the increase in visual acuity was more pronounced and amounted to 74% (from 0.78±0.71 to 0.2±0.3 according to LogMAR, p<0.05), postoperative strabismus and diplopia remained at a high level - 40% of the number of surgical operations performed. The best results of improving visual functions were obtained in patients of the second subgroup with simultaneous TEOD and LBOD (balanced orbital decompression): the increase in visual acuity was 79% (from 0.57±0.47 to 0.12±0.2 according to LogMAR, p<0.05). In patients of this subgroup, strabismus develops less frequently (22% of the number of operated orbits). CONCLUSION: Transnasal endoscopic decompression of the orbit in the inactive form of the disease is indicated for exophthalmos of more than 4-8 mm. In optical neuropathy, transnasal endoscopic orbital decompression is indicated regardless of the initial visual acuity of the patient and should be supplemented with lateral bone orbital decompression with visual acuity below 0.1. The obtained results allowed us to form an algorithm for surgical treatment of patients with endocrine ophthalmopathy and optical neuropathy using transnasal endoscopic orbital decompression as a surgical intervention.

[Nonspecific inflammatory reaction after implantation of palpebral implants]. / Nespetsificheskaya vospalitel'naya reaktsiya posle implantatsii pal'pebral'nogo implantata.

PURPOSE: To identify the possible cause of an inflammatory reaction to a Russian-manufactured palpebral implant made of gold in the long term after surgery, and to determine the clinical and morphological changes in the tissues of the upper eyelid when the presence of the implant caused the inflammatory reaction. MATERIAL AND METHODS: The results of 150 operations with placement of a palpebral implant were analyzed. In 12 cases, a nonspecific inflammatory reaction was revealed within 2 to 4 weeks after the operation, in 7 cases it necessitated explantation, in 5 cases the local long-term use of an ointment with a corticosteroid made it possible to avoid extrusion and explantation. Chemical microanalysis of the palpebral implant and fragments of the removed capsule was performed using scanning electron microscopy (SEM), as well as immunohistochemical (IHC), macro- and microscopic examination of the fragments of connective tissue capsule was carried out after removal of the palpebral implant. RESULTS: The obtained data confirm the chemical purity of the implant, the absence of abnormal accumulation of metals in the tissues of the eyelid. IHC, macro- and microscopic examination of the presented fragments of the connective tissue capsule revealed signs characteristic of an inflammatory reaction to a foreign body. CONCLUSION: Further research is needed to establish the factors and predictors for the development of inflammatory reactions to a foreign body and, in particular, to gold.

[Tarsorrhaphy in rehabilitation of patients with thyroid eye disease]. / Tarzorafiya v sisteme reabilitatsii patsientov s endokrinnoi oftal'mopatiei.

PURPOSE: To evaluate the role of tarsorrhaphy and its effectiveness in the treatment of patients with thyroid eye disease (TED). MATERIAL AND METHODS: The study identified the patients who required tarsorrhaphy for corneal lesions associated with TED among the total number of 457 patients who underwent tarsorrhaphy at the Research Institute of Eye Diseases over the past 20 years. RESULTS: Overall, 477 tarsorrhaphy surgeries (457 patients) were performed, including temporary and permanent, partial and complete. The corneal involvement in TED was the indication for tarsorrhaphy in 81 patients (101 tarsorrhaphies). TED patients were divided depending on the date of operation, and it was found that 61 tarsorrhaphies were performed in the period from 2000 to 2009, and 40 similar interventions were performed in the period from 2010 to 2019. CONCLUSION: The reduction in the number of tarsorrhaphy surgeries in patients with TED in the last decade due to improvement of techniques for bony decompression of the orbit and increase in the number of these operations allowed significant reduction of the degree of proptosis and, as a consequence, the exposure surface area of the eye. However, despite all the successes achieved in orbital surgery, tarsorrhaphy still remains an affordable technique that can always be used in case of corneal lesions in thyroid eye disease.

[Corneal confocal microscopy in Fabry patients with cornea verticillata]. / Konfokal'naya mikroskopiya rogovitsy u patsientov s vikhrevidnoi keratopatiei pri bolezni Fabri.

PURPOSE: To study the changes in corneal microstructure and nerve fibers in patients with cornea verticillata in Fabry disease. MATERIAL AND METHODS: The study included patients with a confirmed diagnosis of Fabry disease. All patients underwent standard ophthalmological examination. Corneal confocal microscopy was performed when slit-lamp examination revealed cornea verticillata. RESULTS: Cornea verticillata was found in 64.5% of patients. Corneal microscopy revealed hyperreflective deposits in the basal cell layer of corneal epithelium in all patients with cornea verticillata. Characteristic features were desquamation of epithelial cells with islands of epithelial cells deficiency, as well as hyperreflective intracellular deposits in the basal layer. Changes in keratocytes in the form of perinuclear white microdots were found in the stroma of 25.8% of patients. Endothelial layer was not changed and had normal cell density, reflectivity and morphology in all patients with cornea verticillata. CONCLUSION: Corneal confocal microscopy is a valuable diagnostic tool in patients with Fabry disease and may be important in evaluation of disease progression and monitoring of treatment efficacy.

[Histological verification of autofluorescence borders of periorbital skin tumors]. / Gistologicheskaya verifikatsiya autofluorestsentnykh granits novoobrazovanii kozhi periorbital'noi oblasti.

Tumor borders are one of the most significant characteristics of any tumor, including that of the skin. PURPOSE: To compare histological borders of periorbital skin tumors with their autofluorescence borders built from the analysis of non-induced protoporphyrin IX autofluorescence. MATERIAL AND METHODS: The study group included 8 patients with skin tumors of the eyelids, periorbital region, eyebrow and zygomatic regions aged 54-88 years. The tumors varied in size from 2 to 8 mm and all displayed signs of basal cell carcinoma (BCC). At admission, all the patients underwent non-induced autofluorescence diagnosis. The images were processed with the «CancerPlot¼ program. During radio excision, the autofluorescent border of each neoplasm was marked with a surgical incision of about 5 mm long and 2 mm deep. RESULTS: Upon pathomorphological examination, solid BCC was identified in 7 cases. The remaining case was senile keratosis. All reference incisions were located in healthy tissues not farther than 1 mm from the tumor (or keratosis locus, correspondingly). CONCLUSION: By the example of facial BCC, an evident correlation was established between histological borders of the tumor and its native (non-induced) protoporphyrin IX autofluorescence.

[Dysthyroid optic neuropathy: surgical treatment potential]. / Opticheskaya neiropatiya pri endokrinnoi oftal'mopatii: vozmozhnosti khirurgicheskogo lecheniya.

PURPOSE: To evaluate the effectiveness of bony orbital decompression in patients with dysthyroid optic neuropathy (DON). MATERIAL AND METHODS: The study analyzed 255 patients with thyroid eye disease (TED) and bony orbital decompression. Those among them who had DON as an indication for surgery were investigated further. Patients underwent standard ophthalmological examination, computer perimetry, color vision assessment using Ishihara tables, relative afferent pupillary defect (RAPD), computed tomography (CT) of the orbit, and in some cases optical coherence tomography (OCT) of the optic nerve. RESULTS: Final analysis included 31 patients (52 eyes). On 13 orbits, only lateral wall decompression was performed, and medial wall decompression was the only intervention in 7 orbits. In other cases, these techniques were performed either simultaneously - in 14 orbits, or alternately - in 18 orbits. In the postoperative period, all patients showed significant positive dynamics in terms of visual acuity, visual field, and proptosis. In all cases, decrease of the amount of orbital inflammation was observed. Exophthalmos significantly decreased after surgery and averaged 20.5±3.1 mm, which is 4.7 mm less than the initial one. All changes were statistically significant (p<0.01). CONCLUSION: Bony orbital decompression is an effective and safe treatment option for DON resistant to high doses of glucocorticoids. In the vast majority of cases, this intervention is the only way to improve and stabilize visual function in this severe category of patients.

[Efficacy of transethmoidal orbital decompression in patients with dysthyroid optic neuropathy]. / Effektivnost' transetmoidal'noi dekompressii orbity u patsientov s opticheskoi neiropatiei pri endokrinnoi oftal'mopatii.

OBJECTIVES: To estimate efficacy and safety of endoscopic orbital decompression in patients with dysthyroid optic neuropathy (DON). MATERIAL AND METHODS: 19 patients aged 54.8±7.1 y.o. with DON were enrolled into the study. RESULTS: Visual acuity was from counting fingers till 0.9 (0.4±0.28) before surgery, it increased till 0.7±0.2 (p<0.05) after surgery. The amount of correct Ishihara plates increased from 5.8±7 till 13.3±7.6 (p<0.05). Exophthalmos reduced by 1.7 mm (p<0.05). Significant reduce of orbital inflammation was noted as well. CONCLUSIONS: This study is the first study of this kind in Russian Federation. Obtained results showed high efficacy and safety of endoscopic orbital decompression in patients with DON.

[Bony orbital decompression in the treatment of endocrine ophthalmopathy]. / Kostnaia dekompressiia orbity v lechenii éndokrinnoi oftal'mopatii.

The article reveiws the problems of bony orbital decompression for thyroid-related orbitopathy. Indications for this kind of surgery and different approaches are discussed. Advantages and limitations of each method are analyzed. Special attention is paid to complications of different bony orbital decompression approaches, complication rate and its efficacy.

[Diffuse choroidal hemangioma as a manifestation of Sturge-Weber syndrome (a clinical case study)]. / Diffuznaia gemangioma khorioidei kak proiavlenie sindroma Sturge-Weber (klinicheskii sluchai).

Encephalotrigeminal angiomatosis (Sturge-Weber syndrome) is a congenital sporadic disease characterized by angiomatosis of meningeal vessels, facial and eye capillaries. The article presents a clinical case of comorbidity between monolateral skin angioma, ipsilateral conjunctival and choroidal angiomas without intraocular pressure elevation. The case is unusual due to asynchronous manifestation of the clinical signs of Sturge-Weber syndrome. Facial angioma was diagnosed at birth, while conjunctival angioma appeared at the age of 45 - at the same time as monolateral hypermetropia, which may be the first symptom of diffuse choroidal angioma.

[IgG4-associated disease in differential diagnosis of inflammatory orbitopathy].

IgG4-associated disease (IgG4-RD) is a systemic inflammatory disease characterized by tumorlike sclerosing masses in different organs. Differential diagnosis in orbital IgG4-RD includes majority of conditions, such as thyroid eye disease (TED), sarcoidosis, granulomatosis with polyangiitis, idiopatic orbital inflammation, limphoproliferative diseases and others. A case of IgG4-RD with different organs involvement and complicated differential diagnosis is presented. This case demonstrates very uncommon manifestation of IgG4-RD, when orbital involvement was very similar with TED. Systemic process was not recognized during a long period of time and diagnosis of IgG4-RD was established only after biopsy of abnormally increased lacrimal gland. Differential diagnosis included other systemic diseases, first of all sarcoidosis, GPA, and lymphoma. Biopsy results were consistent with the gold standard of diagnosis, e. g. more than 40% of plasma cells were IgG4 positive. This case demonstrates the necessity of orbital biopsy before starting immunosuppression to avoid inappropriate treatment strategy.

[Clinical manifestations of orbital lesion in granulomatosis with polyangiitis]. / Klinicheskie proiavleniia porazheniia orbity pri granulematoze s poliangiitom.

One of the most common and potentially threatening manifestations of granulomatosis with polyangiitis (GPA) is orbital lesion. PURPOSE: To study the clinical course and prognosis of orbital lesions of various localization in GPA patients. Material and me-thods. The study included 226 patients with GPA, 74 of them with orbital lesion. Ophthalmic examination consisted of visual acuity test, biomicroscopy, ophthalmoscopy, exophthalmometry and ocular mobility test. Visualization was done using multislice computed tomography and/or magnetic resonance tomography and/or ultrasound examination. RESULTS: Among the patients of the study group, in 35.1% the lesion was limited to dacryoadenitis, in 4.0% of patients - to myositis, and 70.8% had extensive inflammatory orbital masses. Patients with orbital masses had systemic disease in 51.1%, compared to 7.7% in dacryoadenitis (p=0.00). Clinical progression in patients with orbital masses was characterized by severe exophthalmos, periorbital swelling and hyperemia. Patients with dacryoadenitis, on the contrary, had only mild symptoms. Patients with orbital masses had unfavorable prognosis. There were only three patients with myositis in the study group, so the data on them is limited; their clinical symptoms included light periorbital swelling, exophthalmos, strabismus, painful binocular diplopia and eye movement restriction. The disease was recurring in two cases. CONCLUSION: Patients with orbital involvement in GPA may have different course of the disease depending on the localization of inflammation.

Clinical features of different orbital manifestations of granulomatosis with polyangiitis.

PURPOSE: The aim of this study was to distinguish different forms of orbital involvement in granulomatosis with polyangiitis (GPA) and to compare their clinical course and outcomes. METHODS: Two hundred twenty-six consecutive patients with GPA were retrospectively studied. All patients fulfilled the classification criteria of American College of Rheumatology; diagnosis of localized GPA was established according to Chapel Hill Consensus. Of those, 74 patients with orbital disease were enrolled into further investigation of different forms of orbital disease. RESULTS: Overall ocular involvement was diagnosed in 50% (113/226) of patients; in 5.3% (12/226), inflammatory eye disease resulted in permanent visual loss. Most common ophthalmic manifestations were orbital masses, episcleritis/conjunctivitis, and scleritis (74/226, 32/226, and 12/226, respectively). Seventy-four patients with orbital involvement were divided into three groups: those with orbital mass without primary lacrimal gland involvement (1st group, 45 patients), those with lacrimal gland involvement (2nd group, 26 patients), and those with extraocular myositis (3rd group, 3 patients). Symptoms such as orbital pain, decreased vision, double vision, red eye, scleritis, orbital wall destruction, primary gaze strabismus, and ocular motility restriction were significantly more common in the 1st group. Peripheral ulcerative keratitis (PUK) was present in the 1st group only. Patients of the 1st group suffered from visual loss, double vision, primary gaze strabismus, and ocular motility restriction significantly more often. Eleven of them developed visual loss; 5 of them underwent enucleation, whereas in the group of patients with dacryoadenitis, only 1 patient had BCVA less than 0.3. CONCLUSIONS: Three types of orbital disease in GPA were proposed: orbital mass without primary lacrimal gland involvement, dacryoadenitis, and extraocular myositis. Patients with orbital mass without lacrimal gland involvement are characterized with a higher rate of systemic disease, severe clinical course not uncommonly associated with other ophthalmic manifestations (necrotizing scleritis, PUK, orbital walls destruction), relatively unfavorable outcome with high level of morbidity (optic nerve atrophy, anophthalmos, strabismus), and higher rate of recurrences. Dacryoadenitis in GPA showed to be not severe manifestation with favorable prognosis.

Cornea verticillata in Fabry disease.

AIM: To study the frequency of cornea verticillata in patients with Fabry disease and it's relation to the severity of the disease and the types of mutation in the GLA gene. MATERIALS AND METHODS: We studied 69 adult (over 18 years) patients with a classic form of Fabry disease that was confirmed by enzymatic and molecular genetic studies. There were 39 males and 30 females. The median age was 39 years [30.0; 50.0]. The severity of Fabry disease was assessed using the Mainz Severity Score Index (MSSI) with a maximum value of 76 points. Depending on the MSSI score, patients were classified into mild (<20), moderate (20-40), and severe (>40) clinical categories. RESULTS: At least one classic symptom of Fabry disease was present in 88.4% of patients. The majority of patients had the missense mutations of the GLA gene. Cornea verticillata was found in 65.2% of patients and occurred with a similar frequency in males (56.4%) and females (76.7%; p=0.07). Cornea verticillata was the single classic symptom of Fabry disease in only 4.9% of cases, while the rest of the patients presented with angiokeratoma, neuropathic pain and/or hypohidrosis. The frequency of classic symptoms of Fabry disease, as well as renal disease (with the exception of terminal chronic renal failure), brain and heart damage was similar in patients with and without cornea verticillata. Median MSSI scores were also similar in patienths with and without cornea verticillata (20.0 and 18.5 points, respectively; p=0.92). Similar results were obtained in males (26.5 and 30.0 points, p=0.97) and females (16.0 and 16.0 points, p=0.45). The frequency of cornea verticillata did not differ in patients with different types of mutations in the GLA gene. CONCLUSION: Cornea verticillata occured in 65% of adult patients with Fabry disease, was usually accompanied by the other classic symptoms of the disease, and was not associated with the severity of the disease.

[The frequency of ophthalmologic manifestations of granulomatosis with polyangiitis (Wegener's) and their relationship to systemic diseases]. / Chastota oftal'mologicheskikh proiavlenii granulematoza s poliangiitom (Vegenera) i ikh sviaz' s sistemnoi patologiei.

AIM: To estimate the frequency of lesions in the organ of vision in granulomatosis with polyangiitis (GPA) (Wegener's) and to determine their relationship to systemic diseases. SUBJECTS AND METHODS: The retrospective study enrolled 218 patients followed up at the E.M. Tareyev Clinic of Nephrology, Internal and Occupational Diseases, with a diagnosis of GPA. The frequency and association of ophthalmic manifestations with systemic involvement were statistically analyzed using PASW Statistics 18. RESULTS: The organ of vision was impaired in 48.1% of the patients with GPA. The most common manifestations were orbital space-occupying lesion (22.9%), conjunctivitis/episcleritis (14.7%), dacryocystitis (6.0%), and scleritis (4.6%). Orbital space-occupying lesions occurred more frequently in the local type of the disease (p=0.0003), and, on the contrary, the involvement of the conjunctiva and eyeball was seen in patients with the systemic types of GPA (p=0.02). CONCLUSION: The findings may suggest that the orbital lesion is an independent manifestation of GPA, which develops more commonly in its local type. Conjunctivitis/episcleritis is, on the contrary, more frequently seen in the active phase of the disease and generally in the involvement of other organs and systems.

[Combination therapy of refractory localized granulomatosis with polyangiitis and orbital involvement]. / Kombinirovannoe lechenie refrakternoĭ formy granulematoza s poliangiitom s porazheniem orbity.

Granulomatous orbital cellulitis is one of the most common ocular manifestations of granulomatosis with polyangiitis (Wegener's). The process is often refractory to conventional immunosuppressive therapy and requires a more radical treatment approach. However, surgical experience with this type of patients is limited. There have been just a few reported cases of orbital decompression in such patients and many authors have doubted the appropriateness of the procedure, since it is associated with a high risk of potentially fatal complications. Our experience demonstrates the feasibility and effectiveness of orbital mass removal as to pain relief and exophthalmos reduction.

[Surgical rehabilitation of patients with thyroid eye disease: systematic approach]. / , Khirurgicheskaya reabilitatsiya patsientov s endokrinnoi oftal'mopatiei: sistematizirovannyi podkhod.

AIM: to analyze the results of surgical treatment of patients with thyroid eye disease (TED), including the type of surgery, indications, and outcomes. MATERIAL AND METHODS: Hundred thirty-nine patients with TED (112 females, 27 males) aged 45.8±13.6 years. RESULTS: Bony orbital decompression was performed in 91 patients (128 orbits). After the surgery, visual acuity increased from 0.84±0.47 up to 0.94±0.32 (р<0.05), exophthalmos got reduced by the average of 3.4 mm. Strabismus surgery was performed in 15 patients. The absence of diplopia in the primary position of gaze was achieved in 12 cases and the degree of residual heterophoria averaged 3.33 PD (2-6 PD). Ocular motility improved from 10.7° up to 34.58°. Eyelid surgery was performed in 81 patients. CONCLUSIONS: 1. Surgical treatment for thyroid eye disease is indicated in patients with visual impairment and/or significant decrease in the quality of life, if pathogenetic treatment fails. 2. The choice of surgical strategy depends on several factors, such as TED activity and severity, thyroid function, pathologic changes in the orbital tissues, and surgeon's experience and preference. 3. Current surgical techniques for thyroid eye disease enable correction of disease consequences, such as exophthalmos, optic neuropathy, impairment of ocular movement, and exposure keratopathy. 4. Adherence to the systematic approach that includes bony orbital decompression, strabismus surgery, and correction of lagophthalmos and eyelid retraction, ensures high-level functional and cosmetic rehabilitation in patients with TED.

[Refractory form of orbital granulomatosis with polyangiitis: A clinical and morphological study]. / Refrakternaya forma granulematoza s poliangiitom orbity: kliniko-morfologicheskoe nablyudenie.

Orbital granulomatosis with polyangiitis (Wegener's granulomatosis, GPA), which is characterized by granulomatous inflammation with small-vessel vasculitis, can develop in local and generalized forms of the disease. The introduction of current immunosuppressive therapy regimens has improved the prognosis of the disease; however, there are immunosuppressive treatment-refractory forms of GPA, the morphology of which has been inadequately investigated. The paper describes a clinical case of refractory GPA involving the orbit, as evidenced by histological and immunohistochemical examinations. The specific feature of the case is the development of severe fibrosis with the accumulation of mainly type III collagen and the persistence of granulomatous inflammation and productive-destructive vasculitis.

[Core principles of treatment of corneal damage in patients with thyroid eye disease]. / Osnovnye printsipy lecheniya porazhenii rogovitsy pri endokrinnoi oftal'mopatii.

AIM: To develop a therapeutic approach and to estimate the efficiency of complex treatment of corneal damage in patients with thyroid eye disease (TED). MATERIAL AND METHODS: The study enrolled 44 patients (52 eyes) divided into 2 groups depending on the severity of corneal damage. Treatment of those with severe involvement included pathogenetic measures (pulse steroid therapy and/or radiation therapy) and surgery (orbital decompression, eyelid and corneal surgery). RESULTS: As the result of the treatment, orbital inflammation decreased and the state of the cornea improved in all patients. CONCLUSION: The treatment of corneal damage in patients with TED may differ depending on numerous factors, such as the severity of corneal damage and activity of orbital inflammation. Taking into account the potential danger of corneal involvement, one should make efforts to early detection and management of the risk factors.

[Potentials of intraoperative navigation during balanced orbital bony decompression in thyroid eye disease (preliminary results)]. / Vozmozhnosti intraoperatsionnoi navigatsii v sbalansirovannoi kostnoi dekompressii orbity pri endokrinnoi oftal'mopatii (predvaritel'noe soobshchenie).

UNLABELLED: Precise instrument localization is of great importance in orbital decompression surgery. Different navigation systems were designed to fulfill this task and gained wide acceptance. AIM: to describe principal features and determine advantages of intraoperatively navigated orbital bony decompression. MATERIAL AND METHODS: Data on orbital decompression surgery performed with or without intraoperative image guidance is presented. Each time two surgeons were involved - an ophthalmologist (lateral wall decompression) and otolaryngologist (medial wall decompression). RESULTS: In lateral wall decompression, electromagnetic navigation was mostly used to enlarge the created bone window upward and backward, which is particularly challenging in case of bone thinning in the posterior part of its inner rim. In transethmoidal decompression, the image guidance allowed a more extensive removing of the greater sphenoid wing as well as the posterior medial wall where it joins the lateral wall of the sphenoid sinus. As shown by MSCT, lateral rectus muscle dislocation into the created bone window was more significant in navigated procedures. CONCLUSION: This preliminary data convincingly shows advantages and potentials of the use of electromagnetic navigation in orbital decompression surgery, especially, for optic neuropathy in thyroid eye disease patients.

[Ophthalmologic manifestation of granulomatosis with polyangiitis (Wegener's granulomatosis)].

Granulomatosis with polyangiitis (GPA), formerly earlier known as Wegener's granulomatosis, is primary system necrotizing vasculitis of small and medium-sized vessels, which is characterized by the formation of foci of granulomatous inflammation. One of the target organs in GPA is the organ of vision. Its affection may not only reduce quality of life, but also may lead to the severest complications even to fatal outcome. The affection varies in severity from slight inflammation of the ocular superficial structures to severe involvement of all orbital and ocular structures to develop complete blindness, including bilateral one. GPA can affect any portion of the organ of vision. The changes are low specific, which makes early diagnosis and timely adequate therapy difficult. Overall all ophthalmologic manifestations can be divide into 4 groups: 1) lesions of the orbit and appendages; 2) those of the conjunctiva and fibrous tunic of the eyeball: 3) those of the retina: 4) those of the optic nerve. Orbital affection is characterized by the development of granulomas, dacryoadenitis with marked inflammatory infiltration of the surrounding tissues, including oculomotor muscles, and destruction of orbital bony walls. Nonspecific conjunctivitis and episcleritis are commonly encountered. Affection of the fibrous tunic of the eyeball is characterized by the development of necrotizing scleritis, peripheral ulcerative or stromal keratitis. The retina is rarely involved and mainly described as sporadic cases in the literature. The optic nerve is most commonly damaged due to the compression of the orbit by inflamed tissue; the lesion is more infrequently ischemic.