PURPOSE: To describe a case of Wyburn-Mason Syndrome, which is a rare phakomatosis, and its associated optical coherence tomography findings. CASE SUMMARY: A 5-year-old boy was referred to our clinic for decreased vision in his right eye. He had nevus flammeus on his right forehead, upper eyelid, and cheek. His best corrected visual acuity was hand motion in the right eye, in which a relative afferent pupillary defect was noted. Fundus examination of the right eye showed marked dilation and tortuosity of retinal vessels, which could not be discriminated between arteries and veins on the posterior pole. Optical coherence tomography showed multiple large intraretinal vessels without involvement of the internal limiting membrane. Cerebral angiography revealed arteriovenous malformations (AVMs) and feeder vessels from the internal carotid artery to the ophthalmic artery, extending to the ethmoidal artery in the nasal cavity. CONCLUSIONS: Wyburn-Mason syndrome is a rare phakomatoses and shows unique retinal AVM. Misdiagnosis or undertreatment of Wyburn-Mason syndrome can lead to serious complications. Patients with facial nevus flammeus should undergo thorough ocular examinations to rule out this disorder. Optical coherence tomography is a useful method for understanding structural features of retinal vascular malformations in Wyburn-Mason syndrome.
Arteries ; Arteriovenous Malformations ; Carotid Artery, Internal ; Cerebral Angiography ; Cheek ; Child, Preschool ; Diagnostic Errors ; Eyelids ; Forehead ; Hand ; Humans ; Male ; Membranes ; Methods ; Nasal Cavity ; Neurocutaneous Syndromes ; Nevus* ; Ophthalmic Artery ; Port-Wine Stain* ; Pupil Disorders ; Retinal Vessels ; Retinaldehyde ; Tomography, Optical Coherence* ; Vascular Malformations ; Veins ; Visual Acuity

DHA, one of w-3 fatty acids, modulates cell growth or death though the changes of apoptotic signaling in human endothelial ECV304 cells. We investigated the effects of DHA on the changes of apoptotic signaling in human vascular endothelial ECV304 cells using lipid peroxidation (LPO) metabolites. LPO could be originated by dietary polyunsaturated fatty acids such as linoleic acid (LA), arachidonic acid (AA) and docosahexaenoic acid (DHA). DHA caused cell death of ECV304 cells compared to LA, AA or control as evidenced by changes in cell morphology and MTT assay. LPO levels was significantly elevated by 10 fold in DHA-treated ECV 304 cells and caspase-3 activity was increased by DHA corresponding to increasing incubation times compared to control. One of reasons of the cell death in DHA-treated ECV304 cells could be expected that caspase activity, marker for mitochondrial damages, might be triggered by the increasing LPO levels. Our results strongly indicated that DHA induced LPO production has an important role on apoptotic signaling pathway in ECV304 cells. LPO production in endothelial cells which was metabolized by oxidation of dietary PUFA, might be one of risk factors in the initial progression of atherosclerosis.
Apoptosis* ; Arachidonic Acid ; Atherosclerosis ; Caspase 3 ; Cell Death ; Endothelial Cells ; Fatty Acids ; Fatty Acids, Unsaturated ; Humans* ; Linoleic Acid ; Lipid Peroxidation ; Risk Factors

OBJECTIVE: The aim of this study was to evaluate the prognostic significance of primary tumor volume and uterine corpus invasion assessed by MRI in stage II uterine cervical cancer patients treated by concurrent chemotherapy and radiotherapy. METHODS: Fifty-two patients diagnosed with stage II cervical carcinoma were entered into the study. The tumor volume was calculated by the equation (Volume=widthXlengthXheightXpi/6) as an ellipsoid approximation. Univariate and multivariate analyses were performed to identify the prognostic factors for overall survival (OS), disease-free survival (DFS), pelvic control (PC), and distant metastasis-free survival (DMFS). RESUITS: The 5-year OS, DFS, PC, and DMFS rates were 65.8%, 59.3%, 72.6%, and 79.9%, respectively. The average volume of primary cervical tumor on MRI was 29.5 ml (5-109) and volume was not correlated with stage (p=0.180). Corpus invasion was exhibited in 50.0% and 93.8% of patients with small tumor volume (<30 ml) and large tumor volume (> or =30 ml), respectively; and strongly correlated with tumor volume (p<0.001). By univariate analyses, larger clinical tumor diameter (p=0.031), positive pelvic lymph node (p=0.033), uterine corpus invasion (p=0.045), and larger tumor volume (p=0.003) showed a statistically significantly relation to worse survival. In multivariate analyses, dividing patients according to whether the tumor volume was more or less than 30 ml predicted OS (p=0.048) and uterine corpus invasion also predicted DFS (p=0.042). CONCLUSION: Tumor volume and uterine corpus invasion determined by pre-treatment MRI examinations were significant prognostic factors for patients with invasive cervical carcinoma treated with concurrent chemotherapy and radiotherapy.
Disease-Free Survival ; Drug Therapy ; Humans ; Lymph Nodes ; Magnetic Resonance Imaging* ; Multivariate Analysis ; Radiotherapy ; Tumor Burden* ; Uterine Cervical Neoplasms*

PURPOSE: To report the results of preschool vision screening of South Korean children in the year of 2001. METHODS: Nation-wide population-based vision screening tests were conducted in a stepwise manner on the preschool children over 3 years of age. The first step of test was home-screening using 5 familiar picture cards. The children who didn't pass the first step (VA < 0.5 in at least one eye) were sent to the Public Health Care Centers and re-tested there with regular vision charts. After this second step of test, some children were referred to the ophthalmologists. The referral criteria for visual acuity were: at 3 years less than 0.5, at 4 or above 4 years less than 0.63 in at least one eye. Data were collected for the 67, 315 children to evaluate the ophthalmologic conditions. RESULTS: Among 67, 315 subjects who had been enrolled, 97% of children had visual acuity of 0.5 or better. Of those screened, 5, 221 (7.8%) children were referred to the ophthalmologists. The feedback results for 3, 058 (4.6%) children could be collected. Of those referred, 2, 488(80.6%) had one or more ophthalmologic disorders. Refractive errors were found in 2183 children. Amblyopia was discovered in 560 children. Manifest strabismus was detected in 144 children. CONCLUSIONS: In this population setting, ocular disorders which can affect visual development were found in 2, 488 children. This attests to the importance of performing early and effective screening tests for preschool children.
Amblyopia ; Child ; Child, Preschool ; Humans ; Korea* ; Mass Screening ; Public Health ; Referral and Consultation ; Refractive Errors ; Strabismus ; Vision Screening* ; Visual Acuity

PURPOSE: To assess the embolization effect of dural arteriovenous fistula(DAVF). MATERIALS AND METHODS: Weevaluated 23 patients with DAVF who were diagnosed using digital subtraction angiography and treated bytransarterial and/or transvenous emobolization. The locations of DAVFs, as seen on angiography were as follows: 16in the cavernous sinus, four in the major dural sinuses, two in the jugular bulb, one in the torcula Herophili,and one in the tentorium cerebelli. On the basis of venous drainage patterns, Cognard's classification of DAVF wasused. Among our 23 patients, 13 underwent transarterial embolization, six underwent transvenous embolization, andfour underwent both. After embolization, three patients underwent other treatment: surgery, one ; gammairradiation, one ; and both. One. Polyvinyl alcohol particles or glue was used in transarterial embolization, andtungsten, platinum, or Guglielmi detachable coils in transvenous embolization. To analyse the residual lesion inDAF, we compared pre- and post-treatment angiograms ; patients were followed up for 2 to 48 months, and theircondition was assessed as cured, improved, not changed, or aggravated. RESULTS: Nine patients were found to beCognard type I, two were IIa, four were IIa+b, and eight were type III. Of our 23 patients, 12 were cured, tenimproved, and one was aggravated ; of the 13 who underwent transarterial embolization, four were cured, eightimproved, and one was aggravated ; of the six who underwent transvenous embolization, five were cured and oneimproved. The condition of a patient with a lesion in the torcular herophili was aggravated despite surgery andgamma irradiation after embolization. CONCLUSION: DAVF can be managed successfully with endovascular treatment.The outcome of the transvenous approach appears to be better than that of the transarterial approach alone. In acase involving a lesion in the torcular herophili, the outcome was poor and more aggressive treatment wasrequired.
Adhesives ; Angiography ; Angiography, Digital Subtraction ; Arteriovenous Fistula ; Cavernous Sinus ; Central Nervous System Vascular Malformations* ; Classification ; Drainage ; Humans ; Platinum ; Polyvinyl Alcohol

Background: To report the clinical manifestations of non-arteritic anterior ischemic optic neuropathy (NAION) cases after coronavirus disease 2019 (COVID-19) vaccination in Korea. Methods: This multicenter retrospective study included patients diagnosed with NAION within 42 days of COVID-19 vaccination. We collected data on vaccinations, demographic features, presence of vascular risk factors, ocular findings, and visual outcomes of patients with NAION. Results: The study included 16 eyes of 14 patients (6 men, 8 women) with a mean age of 63.5 ± 9.1 (range, 43–77) years. The most common underlying disease was hypertension, accounting for 28.6% of patients with NAION. Seven patients (50.0%) had no vascular risk factors for NAION. The mean time from vaccination to onset was 13.8 ± 14.2 (range, 1–41) days. All 16 eyes had disc swelling at initial presentation, and 3 of them (18.8%) had peripapillary intraretinal and/or subretinal fluid with severe disc swelling. Peripapillary hemorrhage was found in 50% of the patients, and one (6.3%) patient had peripapillary cotton-wool spots. In eight fellow eyes for which we were able to review the fundus photographs, the horizontal cup/ disc ratio was less than 0.25 in four eyes (50.0%). The mean visual acuity was logMAR 0.6 ± 0.7 at the initial presentation and logMAR 0.7 ± 0.8 at the final visit. Conclusion Only 64% of patients with NAION after COVID-19 vaccination have known vascular and ocular risk factors relevant to ischemic optic neuropathy. This suggests that COVID-19 vaccination may increase the risk of NAION. However, overall clinical features and visual outcomes of the NAION patients after COVID-19 vaccination were similar to those of typical NAION.