[An integrated approach to the treatment of pretibial myxedema based on pulse therapy with prednisolone and electrical neuromyostimulation (FREMS-therapy) in a patient with Graves' disease and thyroid eye disease].

Graves' disease (GD) is one of the urgent problems of modern endocrinology, characterized by a high frequency, polysystemic damage to the body, a steadily progressive course, diagnostic difficulties, a high degree of disability and often resistance to therapy. The manifestations of the disease include: thyrotoxicosis syndrome with impaired lipid and carbohydrate metabolism, and activation of multiple organ pathology in the form of thyroid eye disease (TED), pretibial myxedema, cardiovascular insufficiency, acropathy, lesions of the nervous, osteoarticular system, and other lesions. The development of multiple organ pathology can have a different sequence, different time intervals and different degrees of severity. Any developments in the direction of clarifying the etiopathogenetic, clinical diagnostic and treatment-rehabilitation measures are of undoubted significance. We present a clinical case of GD, TED and pretibial myxedema, in which an integrated approach was tested in the tactics of treating pretibial myxedema (a combination of pulse therapy with prednisolone and FREMS-therapy), as a result of which positive results were obtained within a short time.

[Phasing and continuity of the treatment of thyroid eye disease in patients with Graves' disease].

 According to modern concepts, thyroid eye disease (TED) is an independent progressive autoimmune disease of the organ of vision, closely associated with the autoimmune pathology of the thyroid gland (TG), (ICD code - H06.2, proptosis in case of impaired thyroid function E05.0). TED treatment is a long step-by-step process, including immunosuppressive therapy, radiation therapy of the orbits and surgical treatment.TED is a multidisciplinary problem. A patient with thyrotoxicosis clinic and TED symptoms will be taken to an endocrinological clinic for normalization of thyroid hormones and treatment of thyrotoxicosis complications. At the same time, under the supervision of an ophthalmologist, TED diagnostics and treatment will be carried out. Teamwork is of utmost importance because the effectiveness of TED treatment will depend on the speed of achieving a stable euthyroid state, the accuracy of determining the TED activity and severity, and the presence of complications requiring surgical treatment.There are two main phases in the TED development. In the first phase of active inflammation, an increase in the symptoms of TED occurs, then a plateau phase follows, when the symptoms of activity persist but do not progress, then the symptoms regress and the process becomes inactive, while visual disturbances and cosmetic defects may persist. Determining the TED activity is very important from a clinical point of view, because the choice of treatment and tactics of patient management depend on the inflammation activity.We describe a clinical case of phasing treatment of TED complicated by optic neuropathy and movement disorders in a patient with Graves' disease, resistant to immunosuppressive therapy with glucocorticoids and requiring deep lateral bony orbital decompression.

[Ocular involvement in Parry-Romberg syndrome]. / Porazhenie organa zreniya pri sindrome Parri­Romberga.

Parry-Romberg syndrome (PRS) is a rare degenerative disease of unknown etiology, characterized by slow progressive hemifacial atrophy of the soft tissues (subcutaneous fat, muscles), cartilage and bones. Beside hemifacial atrophy, various ophthalmic and periocular manifestations of PRS has been described. Progressive enophthalmos and eyelid changes are only some of many other symptoms, as the disease can affect all layers of the eyeball. This article reviews literature on ophthalmic manifestations of PRS and reports our own case and treatment approach for a patient with this pathology.

[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.

[Experimental study of hyaluronic acid gel fillers biodegradation in orbit]. / Eksperimental'noe issledovanie biodegradatsii fillerov na osnove gelya gialuronovoi kisloty v orbite.

INTRODUCTION: Recently, hyaluronic acid gel (HAG) fillers were proposed as an effective alternative treatment option for surgical orbital volume augmentation. Several authors reported about long-standing effect of the filler. PURPOSE: To assess the features of HAG biodegradation after intraorbital injection in experimental environment. MATERIAL AND METHODS: In the course of the experiment, 7 chinchilla rabbits (14 eyes) received a single 1ml intraorbital HAG injection (Restylane SubQ, Galderma, Sweden) using a cannula. The animals' orbits were examined by ultrasound scan after the injection and at 1, 3, 6, 9, 12 and 18 months. The animals were subsequently sacrificed for morphological study of orbital tissue containing the HAG filler. RESULTS: The HAG filler persisted in the orbit of experimental animals during the whole follow-up period. The volume of HAG depot and its density diminished gradually till the 18th month, after which the particles of HAG could still be detected with the morphological study and ultrasound. CONCLUSION: The HAG filler persisted in the orbit of experimental animals up to 18 months. Incomplete biodegradation explains the longstanding duration of the injection effect.

[Posttraumatic orbital defect correction with bone autografts]. / Ustranenie posttravmaticheskikh defektov glaznitsy s primeneniem kostnykh autotransplantatov.

The aim of the study was the assessment of safety of posttraumatic orbital defect correction with bone autografts. The study comprised 69 patients divided in 2 groups: 21 patients with isolated orbital floor and medial wall deformities and 48 patients with complex midface injuries. Membranous bone autografts were used for deformities correction along with soft tissues hypercorrection with a mix of bone chips and platelet rich plasms. The paper presents diagnostic algorithm for orbital trauma patients. Correction of complex midfacial injuries in group 2 resulted in restricted ocular mobility and increase in diplopy square. Subciliary approach caused lower lid retraction deteriorating esthetic results of the surgery. Ocular vessels hemodynamics showed improvement 1 year after surgery.

[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.

[The complications of transethmoidal decompressive orbitotomy associated with endocrine ophthalmopathy]. / Oslozhneniia transétmoidal'noi dekompressionnoi orbitotomii pri éndokrinnoi oftal'mopatii.

The objective of the present study was the analysis of the results of transethmoidal decompressive orbitotomy (TEDO) with the resection of the bone matrix of the medial and inferior orbital walls in the patients presenting with endocrine ophthalmopathy (EOP) with the purpose of reduction of the risk of development of this pathological conditions. A total of 51 patient with EOP at the stage of complete remission or pharmacotherapeutic compensation underwent transethmoidal decompressive medial orbitotomy in the ENT Clinic of 1 I.M. Sechenov First Moscow State Medical University during the period from 2006 to 2016. Twenty two of these patients presented with the unilateral process and 29 ones with bilateral exophthalmos. The surgical intervention was performed on the total of 80 eye orbits. The main complaints of the treated patients during the post-operative period included difficulties in nasal breathing (33%), dryness of the nose and crust formation (43%), discharge from the nose and along the posterior pharyngeal wall (15%), nasal bleeding (2%), pain in the forehead and maxillary region (8%), headache (2%). As far as the state of eyes and sight is concerned, 14 (27%) patients reported double vision at the primary gaze position while 6 others (12%) complained of the appearance of diplopia upon a change in the gaze position during the post-operative period. The post-operative endoscopic study revealed synechiae of different localization in the nasal cavity (47%), discharge from the maxillary sinuses (16%) and from the frontal sinus (2%). Eight (16%) patients were found to suffer from maxillary sinusitis, one had acute frontitis, and one sphenoidal sinus mucocelle. These complications were eliminated by pharmacotherapy and additional surgical interventions (2%). Analysis of the results of the study has demonstrated that the above complications were associated with the inadequate post-operative care for the nasal cavity, the excessively large size of the bone window formed during transethmoidal decompressive orbitotomy, resection of the inferior orbital wall, intrusion of the medial rectus muscle of the eye into the wide bone window. We evaluated the possible influence of the orbital soft tissues on the physiological parameters of the functioning of the paranasal sinuses in the post-operative period including blockade of the paranasal drainage pathways by the prolapsed orbital tissues. A few possible approaches to the reduction of the risk of post-operative complications are proposed. The authors emphasize the importance of the further improvement of the surgical treatment of endocrine ophthalmopathy.

[Takayasu's disease in an IgG4-positive patient with tuberculosis, pulmonary hemorrhage, recurrent myocarditis, and transient bilateral visual impairments]. / Bolezn' Takayasu u IgG4-pozitivnogo patsienta s tuberkulezom, legochnym krovotecheniem, retsidiviruyushchim miokarditom i prekhodyashchimi dvustoronnimi zritel'nymi narusheniyami.

Takayasu's disease (nonspecific aortoarteritis) affects the absolute majority of 20-40-year-old women; its most common manifestations include aortic arch branch lesions. The specific features of the described case is the male gender of the patient, signs of mesenchymal dysplasia, a concurrence of tuberculosis, pulmonary hemorrhage, and myocarditis at onset, the presence of IgG4, peculiar ocular symptomatology, and its rapid regression after initiation of therapy.

[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.

[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.

[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.