Septo-optic dysplasia (SOD) is a heterogeneous brain midline anomaly associated with ophthalmological, endocrinological, and/or neurodevelopmental symptoms. The clinical phenotype correlates with abnormal brain magnetic resonance imaging (MRI) findings. However, variations of the septum pellucidum (SP) appearance and their clinical impact have not been studied in depth. Sixty-eight patients with optic nerve hypoplasia (ONH) were investigated for the presence of associated SP anomalies and correlations between clinical findings and their MRI abnormalities established. Thirty patients had either complete (n = 22) or partial (n = 8) absence of the SP. Pituitary hormone deficiencies were present in 64% or 25% of the cases, respectively. Neurological symptoms did not occur in patients with SP remnants or unilateral ONH. Hippocampus abnormalities (43%) that have not been described before in SOD and falx abnormalities (17%) correlated significantly with neurological symptoms and developmental delay (p < 0.05 and p < 0.01, respectively). Maternal age at birth was low (21.2 years) and drug abuse during pregnancy was reported in 27% of the patients. Twelve patients with pituitary anomaly and ONH but normal SP showed similar clinical and MRI features, and were classified as SOD-like. The remaining 26 patients were not assigned to SOD. We conclude that unilateral ONH and SP remnants are associated with a milder SOD phenotype. Hippocampus abnormalities and falx abnormalities seem to constitute important features of severe clinical disease, irrespective of SP appearance. Our anamnestic data support the hypothesis of vascular disruption during embryogenesis.

Three males (aged 10 years, 3 years 9 months and 2 years 8 months) with profound sphingomyelinase deficiency are presented. The sphingomyelin storage in the liver biopsies attained 30-fold, 65-fold and 16-fold increases against controls, respectively. Levels of bis(monoacylglyceryl) phosphate were also increased. In two cases the bone marrow contained foam cells with liquid crystals of sphingomyelin. Besides the visceral involvement dominated by hepatosplenomegaly, all three cases showed discrete, so far stationary (8 years, 42 months and 28 months) neuropathic features and retinal lesions resembling the classical cherry-red spot. Electrophysiological examinations showed a variable reduction of peripheral nerve conduction velocity and prolongation of the latencies of somatosensory, visual and auditory evoked potentials. Ultrastructural examination of skin nerves showed a slight storage, mainly in Schwann cells. In some myelinated fibres there were pseudomyelinic ovoids. The cases therefore displayed features of both A and B types of sphingomyelinase deficiency and should be conventionally classified as intermediate. However, the very low levels of in vivo sphingomyelin hydrolysis (not exceeding 6%, against 30 +/- 10% in type B and 77 +/- 5% in controls) were clearly within the range of type A values (5 +/- 2%). Accordingly, we suggest that the cases may be biochemically classified as variants of type A disease.

The CS test by means of the CSV - 1000 device can already detect the first retinal changes in patients with normal visual acuity and is positive in patients with still normal ophthalmologic macular finding.

The authors examined 130 newborns and nursery children from September 1999 till May 2003 from the Prague district for the surmise of chlamydial conjunctivitis. Chlamydia infections were detected in conjunctival smears. Chlamydia trachomatis was confirmed in 20 (15.3%) using ligase chain reaction and C. pneumoniae in 16 (12.3%) children using an indirect immunofluorescent method. Direct captures of chlamydial infections of newborns were included in the study. The authors had also examined 671 newborns in a maternity hospital from January 2002 till May 2003. Conjunctival scraping had been done in 29 (4.3%) cases mainly for mucopurulent conjunctivitis. Chlamydial conjunctivitis was identified only in 4 (0.6%) cases, i.e. C. trachomatis and C. pneumoniae in 2 cases each. Initial clinical symptoms of both types of chlamydial conjunctivitis were similar (mucous discharge with various degrees of eyelid effusion and chemosis mainly on the tarsal conjunctiva). Clinical symptoms of the C. pneumoniae infection were later accompanied by pseudofollicular changes on the tarsal conjunctiva. The complication of this infection was lacrimal obstruction among half of newborns. Clarithromycin in syrup at a dose of 15 mg/kg/per day for 14 days ensured effective treatment of both chlamydial infections. Control scrapings were always negative and simultaneously the pathological conjunctival finding disappeared.

Cíl: Retrospektivní hodnocení prvních 12 měsíců léčby makulárního edému u okluze větve sítnicové žíly (BRVO) preparátem ranibizumab na základě anatomických a funkčních parametrů. Metodika: V hodnoceném souboru bylo zahrnuto 54 očí s makulárním edémem komplikujícím BRVO, s průměrnou vstupní nejlepší korigovanou zrakovou ostrostí (NKZO) 4/16 ETDRS optotypu, průměrnou centrální retinální tloušťkou (CRT) 512,3 µm a průměrnou dobou zařazení do léčby 4 měsíce. Po aplikaci 3 úvodních injekcí 0,5 mg ranibizumabu probíhala léčba v režimu pro re nata, s možností doplnění adjuvantního laseru-mřížkové laserové fotokoagulace (LFK). Retrospektivně byl zhodnocen: počet aplikovaných injekcí, zastoupení očí, kde byla během léčby doplněna mřížková LFK, z funkčních parametrů zisk písmen a nejlepší korigovaná zraková ostrost (výsledná NKZO ≥ 4/8 ETDRS, výsledná NKZO s ohledem na vstupní NKZO) a z anatomických parametrů průměrná CRT a zastoupení očí s CRT ≤ 250 µm, resp. ≤ 300 µm. Výsledky: V celém souboru bylo v prvním roce aplikováno v průměru 4,8 injekcí ranibizumabu. U 42 očí byla provedena mřížková LFK: u 8 očí pouze před zahájením léčby ranibizumabem, u 7 očí před i během léčby, u 29 očí až v průběhu léčby ranibizumabem. Zisk písmen po 12 měsících léčby byl v průměru +16,5 písmen, u 63 % (n=34) očí byl zisk ≥ 15 písmen. Výslednou NKZO ≥ 4/8 ETDRS dosáhlo po 12 měsících léčby 72 % (n=39) očí. Ve skupině se vstupní NKZO ≥ 4/10 ETDRS dosáhlo výsledné NKZO ≥ 4/8 ETDRS 96 % (n=23) očí, u 11 očí byla výsledná NKZO ≥ 4/4. Průměrná CRT po 12 měsících léčby byla 290,3 µm, z toho u 33,3 % očí byla výsledná CRT po 12 měsících léčby ≤ 250 µm, u 64,8 % ≤ 300 µm. Závěr: Při aplikaci relativně nízkého počtu injekcí v prvním roce léčby byla výsledná zraková ostrost na závěr sledovacího období velmi dobrá, potvrdila se i její závislost na vstupní zrakové ostrosti. Rovněž bylo dosaženo i uspokojivých výsledků centrální retinální tloušťky. Klíčová slova: větvová venózní okluze sítnice, makulární edém, ranibizumab SUMMARY RESULTS OF THE FIRST 12 MONTHS TREATMENT OF MACULAR EDEMA COMPLICATING BRVO IN PATIENTS TREATED WITH RANIBIZUMAB The aim of the study: Retrospective evaluation of the first 12 months treatment of macular edema in BRVO with ranibizumab based on anatomical and functional parameters. Methods: 54 eyes with macular edema complicating BRVO were included in the study, with an average initial best corrected visual acuity 4/16 ETDRS charts, an average central retinal thickness 512.3 μm, and in average 4 months till the beginning of treatment with ranibizumab. After 3 initial injections of 0.5 mg ranibizumab treatment was performed in pro re nata regimen, with adjuvant laser (grid laser photocoagulation) as an additional treatment option. The number of injections administered, the number of eyes where grid laser photocoagulation was provided, functional parameters as a letter gain and BCVA (eyes with final BCVA ≥ 4/8 ETDRS, final BCVA in according to initial BCVA) and anatomical parameters as a mean CRT and CRT ≤ 250 μm, respectively ≤ 300...

Aim: Learn about the development and changes in foveal avascular zone (FAZ) and vascularity of retina in the surrounding zone, depending on the duration in young diabetic patients type 1 (T1DM). Methods: As part of regular one-year examinations of young T1DM patients at the Eye Clinic of the University Hospital Královské Vinohrady in Prague (Czech Republic, EU) from January to December 2019, OCT angiography using the device Spectralis (Heidelberg Engineering) was included. Forty patients aged 18 to 30 years were examined, median 21 years. T1DM was diagnosed in childhood and lasted for more than 10 years. At the same time, a control group of forty individuals of similar age, without metabolic and other general disease was examined, normal visual acuity and physiological fundoscopic finding were obligatory. The FAZ size was evaluated in both groups (using built-in function "Draw Region"), also its shape, density decrease and change in character of vascularity of the retina was assessed. Results: In the control group, the FAZ area ranged from 0.06 to 0.4 mm², with an average of 0.253 ± 0.092 mm² and a median of 0.27 mm². It was not affected by a fundamental change in its round shape and the surrounding capillary netting was regular and reasonably dense. In T1DM patients, the FAZ area was in a wider range, from 0.05 to 0.64 mm², an average of 0.300 ± 0.132 mm², and a median of 0.31 mm². The difference in FAZ across-the-board evaluation was statistically significant (p = 0, 009). Diabetic preretinopathy (DpR) was defined by the irregularity of the capillary density up to the manifestation of non-perfusion, in 61% of cases the size of the FAZ was changed. In diabetic retinopathy (DR) there was always an irregularity of the FAZ shape with its enlargement, manifestation of non-perfusion, capillary dilatation and rare microaneurysms. Conclusion: Changes in FAZ size corresponded to the stage of T1DM on the fundoscopic finding of the eye depending on its duration. The initial increased amount of foveal capillaries, which resulted in decreased FAZ area, was followed by a gradual decrease in capillaries and increased FAZ area, consistent with the manifestations of DpR. It was accompanied by a change in capillary density in macula to eventual non-perfusion. On the contrary, the increase in the FAZ area and its irregularity accompanied by non-perfusion of the capillary net and microaneurysms corresponded to the development of DR already.

This study indicated the possible relationship between KCS and Chlamydia pneumoniae in the course of simultaneous clinical signs of follicular conjunctivitis. KCS is a consequence of the action of local infection at the surface of the conjunctiva. It also indicated the necessity of simultaneous evaluation of microbiological findings and the clinical picture in consideration of overall antibiotic treatment in view of the high antibody background of Chlamydia pneumoniae in the adult population in the Czech Republic. The authors aimed to show the possible relationship between the keratoconjunctivitis sicca and Chlamydia pneumoniae based on results of the two studies. Some patents on conjunctivitis are also briefly described in this article.

Aim: The authors assessed the development of intraocular changes in type 1 diabetes (T1DM) from the onset of the disease leading to diabetic retinopathy (DR). The quote: “There must be an intermediate stage between the physiological intraocular finding and the diabetic retinopathy itself “, (prof. Jan Vavřinec). Methods: A two-year study (2018 and 2019) was conducted at the Department of Ophthalmology of the Teaching Hospital Kralovske Vinohrady in Prague (Czech Republic). There were 54 patients aged 17–42 years, the detection of T1DM ranged between the 1st and 14th year of life, with a duration of 12–35 years. Individual patients were always examined simultaneously by three methods: CS (contrast sensitivity), SD-OCT (spectral domain optical coherence tomography) and OCT-A (optical coherence tomography-angiography). We examined 106 eyes once and in a comprehensive manner. Results: We have shown that there is an intermediate stage between the physiological finding on the retina and DR, so-called diabetic pre-retinopathy (DpR). Subsequent redistribution of the observed into two DpR subgroups was derived from the size of the FAZ, either with its smaller area or with a larger area determining the microvascularity of the central area of the retina. The results of both other methods were assigned to these values. For SD-OCT, the depth of the fovea (the difference between the central retinal thickness and the total average retinal thickness) was determined, which was affected by the increased the macular cubature. In all patients it was on average 10.3 μm3. The retina in the central area was significantly strengthened compared to the healthy population at the level of significance p ≤ 0,001. We divided the actual DpR into an image: DpR1 in 26.5 % of eyes – condition with an average shallower fovea only by 21.5 μm below the level of the surrounding retina and an average narrower FAZ: 0.165 mm2 and with a more significant decrease in CS; DpR2 in 40.5 % of eyes – condition with average deeper fovea by 42 μm, i.e., more significantly and average larger FAZ: 0.325 mm2 with lower decrease of CS. At the same time, other changes in microvascularity were noted, such as disorders in the sense of non-perfusion in the central part of the retina of various degrees. This finding differed significantly from changes in already established (non-proliferative) NPDR in 36 % of eyes, when a significant decrease in CS with normal visual acuity was found 4/4 ETDRS. Statistical differences in CS between DpR1 and DpR2 and NPDR were determined – always p ≤ 0.001. The average depth of the fovea was NPDR: 29.5 μm. NPDR had the largest average FAZ: 0.56 mm2. Also significant were the most significant changes in non-perfusion and especially the presence of microaneurysms. Conclusions: These three non - invasive methods helped to monitor the dynamics of the development of ocular changes in T1DM of better quality than the determination of visual acuity and ophthalmoscopic examination. Increased retinal volume induced hypoxia of visual cells with subsequent dual autoregulatory mechanism conditioning two types of diabetic pre-retinopathy before the onset of DR.