RESULTS OF 15 YEARS OF COLLABORATION BETWEEN THE DEPARTMENTS OF OPHTHALMOLOGY AND STOMATOLOGY IN ONCOLOGICAL SURGERY OF THE ORBIT: A DIAGNOSTIC AND THERAPEUTIC APPROACH.

AIM: To report an overview of the most frequent tumors of the orbit, suggest diagnostic approach and possible solution according to experience with own cohort of patients. MATERIAL AND METHODS: From patients files from the Department of Ophthalmology and Department of Stomatology, First Medical Faculty, Charles University, and General Faculty Hospital in Prague, Czech Republic, there were selected patients, who underwent the surgery due to the suspicion of malignant development in the orbit during the period 2005 - 2019. From the surgical records we found information about 497 cases. At the Department of Stomatology, there were 282 surgeries under general anesthesia performed, and at the Department of Ophthalmology, there were 215 surgeries, mostly under local anesthesia performed. RESULTS: The number of surgeries in men and women was equal; patients of all ages were present. The median of patients age operated on at the Department of Stomatology was 53 years, and at the Department of Ophthalmology 63 years. The most common primary benign tumor was the cavernous hemangioma (9 %), the most common non-tumorous expansion was the dermoid cyst (7 %); the most common malignant tumor was the lymphoma (17,5 %). The last mentioned tumor was the most common diagnosis in the whole cohort as well. CONCLUSION: Our cohort of patients is comparable with large cohorts published in the literature concerning age and gender distributions. Differences in frequencies of some lesions may be explained by that our cohort includes patients after the surgery only. The malignant lymphoma is the most common diagnosis indicated to surgical procedure, mostly biopsy. Comparing the two cohorts from our departments 20 years apart, the malignant lymphoma remains the most common indication for surgery, but the incidence of adenomas and adenocarcinomas of the lacrimal gland decreased. It is not the goal of this paper to evaluate all possible orbital affections. Suggested surgical approaches are just recommendations according to years of experience; however, in some situations, to choose an individual surgical approach is necessary.

Ocular Manifestations of Granulomatosis with Polyangiitis.

Granulomatosis with polyangiitis (GPA), formerly known as Wegeners granulomatosis, is an autoimmune vasculitis of small vessels, presenting as necrotizing granulomatous inflammation especially of the upper and lower respiratory tract and necrotizing glomerulonephritis. GPA affects more often Caucasians in northern states, predominantly is affected the age-range group of 50 - 60 years. GPA may affect any organ; the eye symptoms are stated in the range of 16-78 %. The eye symptoms are very variable, and in up to 27 % they are the first sign of undiagnosed GPA. The etiology of GPA was not until now explained. Anti-neutrophil cytoplasmic antibodies (ANCA) play important role in the pathogenesis of this disease. GPA is ranked among ANCA associated vasculitis. The GPA is diagnosed on the basis of clinical signs and symptoms of systemic vasculitis, laboratory and histological tests and imaging studies. Immunomodulative therapy made a contribution to the improvement of GPA prognosis in the last decades; biological treatment reaches the prominence of the GPA treatment procedures. Good collaboration with other specialties is necessary for the early diagnosis and treatment of this life and vision threating disease. The ophthalmologist in the collaboration with specialists of other medical branches may take an important part in the GPA diagnostics, monitoring of the diseases course, or adverse affects of the medication. This paper pays attention to the eye symptoms of the GPA; the literature is supplemented with own photographs of GPA eye symptoms in patients followed up at the Department of Ophthalmology, First medical faculty, Charles University and General Faculty Hospital in Prague, Czech Republic, E.U. Key words: Granulomatosis with polyangiitis (GPA), orbit, scleritis, peripheral ulcerative keratitis (PUK), immunomodulation.

Cognitive sequelae of methanol poisoning involve executive dysfunction and memory impairment in cross-sectional and long-term perspective.

Methanol poisoning leads to lesions in the basal ganglia and subcortical white matter, as well as to demyelination and atrophy of the optic nerve. However, information regarding cognitive deficits in a large methanol sample is lacking. The principal aim of the present study was to identify the cognitive sequelae of methanol poisoning and their morphological correlates. A sample of 50 patients (METH; age 48 ± 13 years), 3-8 months after methanol poisoning, and 57 control subjects (CS; age 49 ± 13 years) were administered a neuropsychological battery. Forty-six patients were followed in 2 years' perspective. Patients additionally underwent 1.5T magnetic resonance imaging (MRI). Three biochemical and toxicological metabolic markers and a questionnaire regarding alcohol abuse facilitated the classification of 24 patients with methanol poisoning without alcohol abuse (METHna) and 22 patients with methanol poisoning and alcohol abuse (METHa). All groups were compared to a control group of similar size, and matched for age, education, premorbid intelligence level, global cognitive performance, and level of depressive symptoms. Using hierarchical multiple regression we found significant differences between METH and CS, especially in executive and memory domains. METHa showed a similar pattern of cognitive impairment with generally more severe executive dysfunction. Moreover, all METH patients with extensive involvement on brain MRI (lesions in ≥2 anatomical regions) had a more severe cognitive impairment. From a longitudinal perspective, we did not find any changes in their cognitive functioning after 2 years' follow-up. Our findings suggest that methanol poisoning is associated with executive dysfunction and explicit memory impairment, supposedly due to basal ganglia dysfunction and disruption of frontostriatal circuitry proportional to the number of brain lesions, and that these changes are persistent after 2 years' follow-up.

[STORY of the Papilla - a Case Report]. / Príbeh jedné papily.

PURPOSE: To present a case report with "unclear" and sudden decrease of left eye visual acuity and bilateral visual fields defects. METHODS: A case report. CASE PRESENTATION: A 66-year-old woman was referred to our Center of Neuroophthalmology and Orbitology by a neurologist for a history of sudden decrease of visual acuity of her left eye 3 years ago. From September 2009, she was examined at various and not only ophthalmology departments. One by one the optic nerve neuritis, traumatic, compressive or toxic neuropathy and also nutritive neuropathy because of vitamin B(12) deficiency were excluded. The patient underwent also a genetic examination for Lebers hereditary optic nerve neuropathy, but this diagnosis was not confirmed. On magnetic resonance imaging, an atrophy of both optic nerves was described, with no further progression found during the follow-up examination after one year. In available patients medical records we found out that on optical coherence tomography scans optic disc drusen of the both eyes are visible, but this wasnt described in the records. Also, an examination of Visual Evoked Potential was performed - this confirmed the diagnosis of optic disc drusen. However, our patient was further examined for visual lost of the left eye. At the time of presentation (January, 2014), her best-corrected visual acuity of the right eye was 0.5, and counting fingers at 50 cm distance with correct light projection in the left eye. Static perimetric examination demonstrated bilateral and concentric narrowing of visual fields. The eyes were parallel, with no limitation of their movements in any direction. The patient was without diplopia, the direct pupil reactions to the light were sluggish bilaterally, and anterior segments of both eyes were with no pathologies. Examination of the fundus revealed bilateral findings of pale optic disc with absent optic cup and indistinct "lumpy" margins. Waxy pearl-like irregularities of the papila of both eyes were visible even without pupil dilatation. Bilateral optic disc drusen were confirmed by ultrasonography, fundus autofluorescence and spectral-domain optical coherence tomography. CONCLUSION: Optic disc drusen are often asymptomatic, frequently it is an accidental finding during the biomicroscopy of fundus due to ordinary eye examination. Rarely, optic disc drusen can cause blood circulation failure on the optic disc with typical defects of the visual field. Thats why we shouldnt forget the optic disc drusen in the differential diagnosis considerations.

[Clinical findings in members of a Czech family with retinitis pigmentosa caused by the c.2426_2427delAG mutation in RPGR]. / Klinické nálezy u clenu ceské rodiny s retinitis pigmentosa podmínenou mutací v ORF15 genu RPGR.

PURPOSE: To describe the phenotype of members of the first Czech retinitis pigmentosa family with an identified molecular genetic cause (c.2426_2427delAG in RPGR), followed for more than 13 years. METHODS: Medical records were reviewed and a detailed ophthalmic examination including spectral-domain optical coherence tomography and full-field and multifocal electroretinography (ERG) was performed in two affected males, three female carriers and one unaffected female. RESULTS: A 22-year-old male who denied suffering from nyctalopia had a best corrected visual acuity (BCVA) of 0.63 in both eyes. Moderate myopia and myopic astigmatism were present bilaterally. Color vision and contrast sensitivity were normal. There was an eccentric constriction of the visual fields that spared the central 20 degrees in both eyes. Fundus examination revealed bilateral pigmentary changes in the mid-periphery. Full-field ERG documented a 10% rod and 20% cone response. The phenotype of his cousin, also aged 22 years, was more severe. He complained of nyctalopia since 12 years of age. His BCVA was 0.3 in the right eye and 0.5 in the left eye. Myopia and astigmatism were present bilaterally. Contrast sensitivity and color vision were severely impaired. Full field ERG was extinct, but some activity on multifocal ERG was still detectable. The constriction of the visual fields reached 5 degrees in both eyes. Fundus examination showed the typical retinitis pigmentosa appearance. All carriers denied that they suffered from nyctalopia, but two of them had decreased BCVA in at least one eye. None exhibited typical bone spicules or a tapetal-like reflex. Significant refractive errors were present in all eyes of the carriers. CONCLUSION: The finding of moderate or high myopia and astigmatism in males with retinitis pigmentosa as well as refractive errors in female relatives indicates possible X-linked inheritance, which may be especially important in pedigrees where the transmission pattern can not be clearly established. Our study highlights the inter-individual variability in phenotype observed in similar aged patients with identical ORF15 RPGR mutations.

[Infliximab in the treatment of Wegener's granulomatosis: case report]. / Infliximab v lécbe Wegenerovy granulomatózy: kazuistika.

PURPOSE: to evaluate the efficacy of monoclonal anti-tumor necrosis factor alpha antibody infliximab in treatment of refractory Wegene's granulomatosis. Clinical manifestations of Wegener's granulomatosis at the time of infliximab initiation were: proptosis of both eyes due to progressive retrobulbar granulomas, visual impairment, optic disc edema, progressive diplopia, sinus and renal involvement. The disease was refractory to a treatment with various immunosuppressants, including intravenous cyclophosphamide or high doses of mycophenolate mofetil in combination with corticosteroids. METHODS: Observational case report. RESULTS: the patient received six infusions of infliximab (3 mg/kg) in addition to low doses of oral cyclophosphamide and corticosteroids. The interval between the first two infusions was 1 week. The interval between the second and third infusions was 4 weeks, thereafter 8, 12 and 34 weeks. Following the third infusion of infliximab the remission of Wegener's granulomatosis was seen. After the regression of retrobulbar granulomas visual acuity increased, ocular motility improved, diplopia and optic disc edema decreased. Remission has been maintained for 17 months. Infliximab was well tolerated during the short-term follow-up. CONCLUSION: Infliximab successfully induced remission in case of sight-threatening Wegener's granulomatosis in our patient. It seems that infliximab provides more effective therapeutic option in treatment of Wegener's granulomatosis refractory to standard immunosuppression and offers better perspectives for patients with previously poor prognosis. Furthermore, it enables reducing the exposure to immunosuppressive drugs.

[Treatment of uveal effusion syndrome after filtering surgery (2 case reports)]. / Chirurgická lécba syndromu uveální efuze na oku po filtrující operaci (2 kazuistiky).

The purpose of this article is to report the surgical treatment of two patients. After the filtering surgery a dehiscence of the conjunctiva emerged and was followed by an extreme hypotony of the eyeball and an extensive uveal effusion. The first patient, 82 years old lady, was treated with the diagnosis of chronic open angle glaucoma for 25 years. The right eye with an absolute glaucoma, the left eye had normal intraocular pressure (IOP) and was after a combined filtering surgical procedure with phacoemulsification and posterior chamber intraocular lens (PC IOL) implantation in 1998. In autumn 2000, the patient suffered conjunctivitis, and the prominent filtering bleb was damaged due to frequent rubbing of the eye because of itching. Severe hypotony of the eyeball with secondary uveal effusion followed. Considering the age of the patient and the only functional eye, an extreme surgery was indicated: PC IOL explantation (removal), pars plana vitrectomy (ppV) with silicone oil implantation. After the surgery the eyeball was anatomically stabilized and the VA (visual acuity) improved from initial uncertain light perception to 0.08 (1/12 or 3/36) and certain light perception. The second patient, 35 years old man suffered in 1995 a serious penetrating injury with an intraocular foreign body of the right globe. During the following five years the patient underwent in altogether six intraocular surgeries due to posttraumatic cataract and rhegmatogenous and tractional detachment of the retina. In the year 2000, trabeculectomy due to secondary glaucoma was indicated and 8 months later, on patients demand an anterior chamber intraocular lens (AC IOL) was implanted. During the first week after the surgery severe hypotonia of the globe and secondary uveal effusion caused by conjunctival dehiscence in the upper nasal quadrant of the eyeball developed. Because of complicated ocular history of the patient who underwent on the whole eight eye surgeries a relative minimal procedure was indicated: AC IOL removal and insufflation of 1 ml of 100% hexafluorosulfide (SF6) expansive gas into the anterior chamber and vitreous cavity. The globe was stabilized and the VA (visual acuity) improved from initial uncertain light perception to 0.25 (6/24 or 20/80) with certain light perception.

[Initial experience with surgical decompression of the vein in branch retinal vein occlusion]. / První zkusenosti s chirurgickou dekompresí zíly u vetvové retinální okluze.

Due to the development of the armamentarium and surgical techniques, the indications of pars plana vitrectomy (PPV) are expanding. It concerns also a relatively new indication, as is the branch retinal vein occlusion (BRVO) of the temporal part of the posterior pole of the retina with the secondary involvement of the macular region. The purpose of this prospective study is to demonstrate anatomical and functional results after the surgical decompression of the occlusion of the branch retinal vein draining the macular region. Since November 1999, 3 patients, one woman and 2 men (age 70, 47 and 76 years) with the quadrant BRVO of the temporal part of the retina involving the macular region were followed. The visual acuity before the surgery in all 3 patients was 6/36 (20/120 or 0.16) and the BRVO lasted for 3-5 months. The patients were examined by slitlamp biomicroscopy, and the course of the disease was documented by means of red-free fundus photographs as well as fluorescein angiograms. In all three patients, PPV and the adventitial sheathotomy of the arteriovenous crossing (AVC) were performed. Surgical decompression of BRVO and reperfusion of the ischemic part of the retina was achieved by separating the overlying arteriole from the venule at AVC. Neither per- nor postoperative complications were noted. In two patients, the visual acuity improved to 6/12 (20/40 or 0.5), and in the last one stabilized at 1/9 (20/180 or 0.11). The minimal follow-up period was 12 months. In conclusion, we recommend to performing this new type of delicate surgery procedure in individual cases on the basis of detailed biomicroscopial examination and fluorecein angiograms by an experienced vitreoretinal surgeon.

[Vitrectomy in optic nerve disc pit with maculopathy]. / Vitrektomie u jamky terce zrakového nervu s makulopatií.

There is described a rare case of a congenital optic disc pit of the 20-years old man that was accompanied by a cyst-like change in the macula with serous retinal detachment. Visual impairment in his right eye was treated with pars-plana-vitrectomy with surgical posterior vitreous detachment and gas tamponade. After temporary pneumatic displacement of the subretinal fluid inferiorly the corrected visual acuity improved to 6/9. Schisislike changes persisted in the macular area. Our findings could support the view on the two-layer structure of optic disk pit maculopathy and an important role of tangential vitreous traction in the pathogenesis of the central retinal detachment.

[Retinal detachment with retinal tears--clinical characteristics and surgical results]. / Odchlípení sítnice s orální trhlinou--klinická charakteristika a operacní výsledky.

The authors evaluate a group of 53 eyes of 52 patients operated on account of retinal detachment with an oral dialysis. The mean follow-up period was 35 months. There were 37 (70%) traumatic dialyses. They were in the temporal lower quadrant in 58%, in the nasal upper quadrant in 16%, temporal upper quadrant in 13% and in the nasal lower quadrant in 13%. There were 16 (30%) idiopathic dialyses, all with one exception in the temporal upper quadrant, they were in the lower temporal quadrant. 52 eyes were operated primarily cryosurgically, one eye by pars plana vitrectomy. After primary surgery the retina adhered completely in 94% eyes. On account of relapses of detachment 9.4% eyes were reoperated. At the end of the follow up the retina adhered completely in 94% eyes. Functional improvement or stabilization of vision was achieved in 78% eyes with traumatic tears and in 87% eyes with idiopathic tears. The authors confirmed the existence of two different clinical entities--traumatic and idiopathic oral dialysis which differ as to site and to a certain extent also the prognosis of the disease.

[Vitrectomy and autologous thrombocyte concentrate in the treatment of idiopathic macular holes]. / Vitrektomie a koncentrát autologních trombocytu v lécbe idiopatických makulárních der.

OBJECTIVE: To evaluate the results of surgery of idiopathic macular holes (IMH) using or not using autologous thrombocyte concentrates (ATC). METHOD: Fifty eyes with IMH, stage 2-4, were divided into two groups. Group 1 comprised 27 eyes with an average persistence of complaints of 12 months where pars plana vitrectomy (PPV) and tamponade with 16% C3F8 were combined with the use of ATC. Group 2 comprised 23 eyes with a mean duration of complaints of 14 months which were treated by PPV and 16% C3F8 without ATC. In all IMH stage 2 and 3 the posterior vitreous membrane was detached from the retina. The epimacular membrane was removed, if present. The mean follow-up period in group 1 was 17.2 months and in group 2 12.3 months. RESULTS: In group 1 anatomical success was achieved in 21 eyes (78%), visual acuity improved by two lines and more in 16 eyes (59%). In group 2 anatomical success was recorded in 15 eyes (69%) (p = 0.326) and visual acuity improved in 10 eyes (44%) (p = 0.265). Nine eyes were reoperated with ATC. Reoperations improved the anatomical success in the whole series to 90% and the functional success to 62%. The visual acuity improved in 70% operated eyes within 12 months from the onset of complaints and in 47% eyes with a longer case-history (p = 0.1182). Complications included retinal tears in 5 eyes (10%), detachment of the retina in one eye (2%), minor facete-like defects in the pigmented epithelium in 22 eyes (44%), disciform maculopathy in 5 eyes (10%) and atrophy of the optic disc in 3 eyes (6%). CONCLUSIONS: PPV is beneficial in IMH in the majority of patients. ATC can improve anatomical and functional results. Functional success is more likely after surgery in the early stage of the disease. The results of surgery are jeopardized by various complications. It is important to inform patients on the limitations and risk of surgery.

Results of penetrating keratoplasty in bullous silicone oil keratopathy.

BACKGROUND: To evaluate the results of penetrating keratoplasty (PK) in bullous silicone oil keratopathy (BSK). MATERIAL AND METHODS: PK for BSK was performed in 13 patients, aged from 31 to 82 years. In all patients, pars plana vitrectomy (PPV) with silicone oil implantation (SOI) had been previously performed as the definitive surgery: for complicated retinal detachment with advanced proliferative vitreoretinopathy in 12 eyes and for diabetic traction detachment in one eye. Seven patients were monocular. All 13 eyes were aphakic and there was a long-lasting contact of silicone oil with the corneal endothelium. Before keratoplasty, each eye underwent between two and five operations, on average three, for retinal detachment or late complications of SOI. The interval between SOI and PK was from 11 to 79 months. The patients were followed up for 12-52 months. RESULTS: At the last follow-up examination, a clear graft was found in 6 out of 13 eyes (46%). The six clear grafts were found in the ten eyes where silicone oil had been removed before keratoplasty. The graft became opaque in all three eyes in which silicone oil had not been removed. Six out of seven eyes with failed grafts atrophied. Visual acuity improved in all six eyes with the clear graft from had movement preoperatively to 0.05-0.25. The decreased visual acuity was caused by irreparable damage to the macula, which resulted from high myopia, proliferative vitreoretinopathy or long-lasting detachment of the macula. CONCLUSIONS: PK in BSK was successful only in those eyes in which the attached retina enabled the removal of the silicone oil before the PK. The reattached retina and useful visual acuity before the development of BSK were the preconditions for functional success of the PK.

[Long-term results of pars plana vitrectomy and silicone oil in large retinal tears in children]. / Dlouhodobé výsledky pars plana vitrektomie a silikonového oleje u obrovských trhlin sítnice detského veku.

Pars plana vitrectomy (PPV) with implantation of silicone oil (ISO) was performed giant tears (GT) in 24 eyes of 22 children aged 6-15 years. In 15 eyes of 13 children idiopathic GT were involved (71%), in 9 eyes (29%) the GT was preceded by an injury-contusion in 5 eyes and a perforating injury in 4 eyes. The patients were followed up for 24-132 months after operation, on average for 55 months. The final anatomical success with an attached macula and functional success with a visual acuity of 0.02 or better were achieved in 18 eyes (75%). The results did not depend on the size of the GT and were better in post-traumatic than in idiopathic GT. The success of the operation declined from 82% after two years and 79% after 3 years to 45% after 5 and more years. The silicone oil was removed in 13 years (46%). Of those in 4 eyes (31%) the detachment relapsed and in two eyes (15%) the relapse was the cause of the final failure. PPV with ISO was a very effective operation in GT. Proliferative vitreoretinopathy was the main cause of surgical failures and a frequent obstacle for removal of silicone oil from the eye. Dispensarization, prophylactic cryopexy and early diagnosis of GT are the main prerequisites of a better prognosis and results in GT in children.

[Heparin-associated type II thrombocytopenia as a cause of multiple thromboembolism complications]. / Heparin-assoziierte Thrombozytopenie Typ II als Ursache multipler thromboembolischer Komplikationen.

13 days after hysterectomy and subcutaneous treatment with unfractionated heparin (10000 IU daily) a 68 year old women developed a pulmonary embolism and deep vein thrombosis of the right leg. She thereupon received intravenous heparin (1000 IU/h). Eight days later she developed acute ischaemia of both legs, and Doppler examination revealed acute Leriche's Syndrome with thrombosis of both iliac arteries. Platelet count fell from, initially 152 x 10(9)/I, to 44 x 10(9)/I. Although heparin-associated thrombocytopenia type II was suspected a confirmation by demonstrating a heparin dependent antibody with the heparin-induced platelet activation (HIPA)-test failed and therefore crossreactivity of low molecular heparins or heparinoids could not be assessed. After discontinuation of heparin and iliacal artery thrombectomy a combination therapy with aspirin plus ticlopidine (500 mg/d respectively) was started and continued until phenprocoumon could exert its full effect. No recurrent thromboembolic events occurred, the platelet counts normalized and the patient fully recovered.