The present report describes a case of VATER syndrome with situs inversus viscerum totalis in a newborn Korean male infant who died immediately after his birth. The disposition of all the organs in thoracic and abdominal cavities formed a complete mirror picture of normal disposition. In addition, the following abnormalities were also present : 1) Unsegmented bar of the 12th thoracic vertebra fused with 11th thoracic vertebra at left side and 1st lumbar vertebra at right side, 2) Imperforated anus (Type 3 of Ladd and Grorss classification), 3) Tracheoesophageal fistula with upper esophageal atresia, 4) Patent ductus arteriosus connected to the descending aorta, 5) Atrial septal defect, septum primum type, 6) Slightly enlarged ureter, 7) Translocation of cecum with appendix beneath the liver, 8) Coiled appendix, 9) Incomplete oblique fissure of the right lung.
Abdominal Cavity ; Anal Canal ; Aorta, Thoracic ; Appendix ; Cecum ; Ductus Arteriosus, Patent ; Esophageal Atresia ; Heart Septal Defects, Atrial ; Humans ; Infant* ; Infant, Newborn* ; Liver ; Lung ; Male* ; Parturition ; Situs Inversus* ; Spine ; Tracheoesophageal Fistula ; Ureter

The microform cleft lip is the mildest expression of cleft lip and nose deformity, but it has no specific definition, classification, and few methods have been reported for its correction. It is characterized by deformity of the nostril, skin striae of the upper lip, notching of peaked Cupid's bow, deformity of the vermilion, and anomaly of the upper lateral incisior and alveolar ridge on the affected region. Sixty-three microform cleft lip patients were operated on between Dec. 1993 and Sep. 1998 in our department(29 males and 34 females). The age of the patients ranged from 5 months to 30 years(Mean 9 years). We classified and treated the microform cleft lip as follows: Class I: Cleft lip nose with very slight lip deformity Class II: Minimal lip deformity without vermilion notching Class III: Mild lip deformity with slight vermilion notching. The goals in the correction of a microform cleft lip are to obtain an esthetically pleasing upper lip and nose, and to reestablish muscle continuity for improved function. To attain these goals, we used the above classification and satisfactory results were obtained by treating the microform cleft according to the classification.
Alveolar Process ; Classification* ; Cleft Lip* ; Congenital Abnormalities ; Humans ; Lip ; Male ; Microfilming* ; Nose ; Skin

No abstract available.
Nitric Oxide*

PURPOSE: To determine whether acute or subacute attack influences the peripapillary atrophy in patients with primary angle-closure glaucoma (PACG). METHODS: Thirty-three eyes with PACG that had an acute or subacute attack (symptomatic PACG group) and 30 eyes with chronic PACG that had no attack (asymptomatic chronic PACG group) were studied. The rim/disc area ratio were matched in both groups. There were no statistically significant differences in the age, sex, refractive error, optic disc area, and visual field mean deviation between the two groups. RESULTS: Nineteen of 33 eyes (57.6%) with symptomatic PACG group and 20 of 30 eyes (66.7%) with asymptomatic chronic PACG group had zone beta. The mean zone beta/disc area ratio was 0.31 in symptomatic PACG group and was 0.52 in asymptomatic chronic PACG group. There were no statistically significant differences regarding the prevalence of zone beta (P=0.63) and the zone beta/disc area ratio (P=0.09) between the two groups. CONCLUSIONS: These findings suggest that the peripapillary atrophy in PACG is almost independent of acute or subacute attack.
Atrophy* ; Glaucoma, Angle-Closure* ; Humans ; Prevalence ; Refractive Errors ; Visual Fields

This study was undertaken to evaluate how closely peripapillary atrophy(PPA) is related to structual and functional optic nerve damage in primary open-angle glaucoma. Magnification-corrected morphometry of photographs using computer graphic program and automated static threshold perimetry were performed on 234 eyes of 141 patients with primary open-angle glaucoma and 139 eyes of 86 normal subjects(control). For the data analysis, only one eye of each patient was randomly selected. Both groups did not differ significantly in age, refractive error and disc area. PPA differentiated into two different zone(alpha and beta). The area of zone alpha, zone beta and the total PPA enlarged significantly)P=0.0001) with a decreasing rim/disc area ratio(r=0.36, r=0.46, r=0.50, respectively), rim area(r=-0.27, r=-0.44, r=-0.43, respectively) and mean deviation(r=-0.30, r=-0.34, r=-0.39, respectively). They enlarged significantly(P=0.0001) with an increasing vertical(r=0.35, r=0.42, r=0.46, respectively) and horizontal cup to disc ratio(r=0.31, r=0.37, r=0.41, respectively) and cup area(r=0.36, r=0.41, r=0.46, respectively). Concerning peripapillary parameters, the correlation coefficients were generally lowest for zone beta and the highest for total PPA. The peripapillary atrophy showed a spatial relation to neuroretinal rim loss. These results indicate that enlargement of PPA is related to the severity of the glaucomatous optic nerve damage and visual field defects.
Atrophy* ; Computer Graphics ; Glaucoma, Open-Angle ; Humans ; Optic Nerve* ; Refractive Errors ; Statistics as Topic ; Visual Field Tests ; Visual Fields

Generalized narrowing of the retinal vessels has been recognized for glaucomatous and nonglaucomatous optic neuropathies. To evaluate how closely peripapillary retinal vessel diameters were related to functional and structural optic nerve damage in primary open-angle glaucoma, the data of the vessel diameters were correlated with intra- and peripapillary morphometric parameters and visual field indices. The diameters of the superior temporal and inferior temporal retinal artery and vein were measured at the opticdisc border from optic disc photographs of 234 eyes of 141 patients with primary open-angle glaucoma and 139 eyes of 86 normal subjects. Only one eye per patient and subject was taken for statistical analysis. The diameters of the inferior temporal and superior temporal retinal artery were significantly correlated with the intrapapillary parameters, the peripapillary parameters and the visual field indices. The correlation coefficients were highest for the neuroretinal rim data (r>0.37, p=0.0001), followed by mean deviation, vertical cup to disc ratios, whereas total peripapillary atrophy data, zone beta and zone alpha data were relatively low. Concerning the vessel diameter, the highest correlation coefficients were calculated for the inferior temporal artery, followed by the superior temporal artery. No correlation was found with age and refraction. The results indicate that, in primary open-angle glaucoma, the vessel diameter redduces with decreasing area of the neuroretinal rim, increasing visual field defects and increasing peripapillary atrophy. Its evaluation can be helpful for the diagnosis of glaucoma and possibly also for follow-up.
Atrophy ; Diagnosis ; Glaucoma ; Glaucoma, Open-Angle ; Humans ; Optic Nerve Diseases ; Optic Nerve* ; Retinal Artery ; Retinal Vessels* ; Retinaldehyde* ; Temporal Arteries ; Veins ; Visual Fields

Narrowing of the retinal vessels in chronic glaucoma has been recognized only recently. We performed this study to evaluate the vessel diameter in normal and glaucoma eyes, addressing whether the retinal vessel diameters differ with the degree of glaucomatous optic nerve damage. The diameters of the superior temporal and inferior temporal retinal artery and vein were measured at the optic disc border from optic disc photographs of 234 eyes of 141 patients with primary open-angle glaucoma and 139 eyes of 86 normal subjects. The photographic magnification was corrected according to Littmanns method. Only one eye per patient and subject was taken for statistical analysis. According to the neuroretinal rim/disc area ratio, the glaucoma group was divided into four stages(early; more than 0.61, medium; 0.60~0.41, advanced; 0.40~0.21, far advanced; less than 0.20). In the normal group the diameter of the inferior temporal vein(0.130+/-0.020mm) was the largest, followed by the superior temporal vein(0.117+/-0.017mm), the inferior temporal artery(0.102+/-0.016mm), finally the superior temporal artery(0.093+/-0.012mm). The retinal vessel diameter decreased significantly with decreasing of the neuroretinal rim/disc area ratio. In the glaucomatous eyes as compared to the normal eyes, the diameters of the inferior temporal and superior temporal retinal artery were significantly smaller at the early and medium stages(p<0.03, p<0.02, respectively). Whereas both inferior temporal and superior temporal retinal vein diameters were significantly samller at the far advanced stage(p=0.01, p=0.005, respectively). The results indicate that generalized reduction of the retinal vessel diameter throughout the retina is related to the severity of glaucoma. From a diagnostic point of view, evaluation of artery diameter rather than vein diameter may be helpful for the differentiation between normal and glaucomatous eyes.
Arteries ; Glaucoma* ; Glaucoma, Open-Angle ; Humans ; Optic Nerve ; Retina ; Retinal Artery ; Retinal Vein ; Retinal Vessels* ; Retinaldehyde* ; Veins

The relationship between quantitative structual measurements of the optic nerve head and visual field defects was studied in 150 eyes of 150 patients with primary open-angle glaucoma(POAG). The radius and angle of the optic disc and cup were measured every 30 degrees to obtain rim area, rim area to disc area ratio, cup area, and cup to disc ratio with a computer graphic program(Adobe PhotoshopTM) and the photographic magnification was corrected according to Littmann`s method. The visual field indices including mean deviation(MD) and corrected pattern standard deviation(CPSD), using a Humphrey Field Analyzer(C 302) were obtained. Each intrapapillary parameter showed statistically significant linear correlation with MD. And those disc parameters also significantly correlated with CPSD, although this correlation was relatively lower than that of intrapapillary parameter with MD. Rim area to disc area ratio and MD were most highly correlated(r=0.712, p=0.0001), and correlation of rim are with MD followed next(r=0.670, p=0.0001). These findings suggest that intrapapillary parameters in POAG were more correlated with the overall visual field depression(MD) than the localized visual field change(CPSD). The rim area to disc area ratio rather than the rim area itself, was more related to the diffuse visual field defects.
Computer Graphics ; Glaucoma, Open-Angle* ; Humans ; Optic Disk ; Radius ; Visual Fields*

Retinopathy in systemic lupus erythematosus consists of cotton-wool spots with or without intraretinal hemorrhages. Although rare, a more severe retinal vasoocclusive disease, termed retinal vasculitis, has been described. The predominant neuropathologic findings are destructive and proliferative changes in the arterioles and capillaries with microinfarcts and occasional foci of inflammatory cells. The authors experienced a case of SLE which was associated with severe retinal vaso-occlusive disease occuring in both eyes of a 31 year old female patient. We report a case of SLE which revealed a typical vaso-occlusive retinal lesion.
Adult ; Arterioles ; Capillaries ; Female ; Hemorrhage ; Humans ; Lupus Erythematosus, Systemic* ; Retinal Vasculitis ; Retinaldehyde*

PURPOSE: To evaluate the outcome and factors that influence the outcome of trabeculectomy with adjunctive mitomycin C in eyes with medically uncontrolled neovascular glaucoma. METHODS: The records of 38 eyes who underwent mitomycin C trabeculectomies were reviewed. Success was defined as intraocular pressure of 21 mm Hg or less with or without glaucoma medications and no additional glaucoma surgery, phthisis, or loss of light perception. RESULTS: Postoperative success was obtained in 22 (57.9%) out of 38 eyes after a mean follow-up period of 18.0+/-18.9 months. The success rate was 57.9% at 1 year and 51.4% at 2 years. The main causes for failure were inadequate IOP control in 34.2% (13/38), loss of light perception in 23.5% (8/34), and progression to phthisis bulbi in 2.6% (1/38). Age of 50 years or younger was a significant risk factor for surgical failure (hazard ratio, 10.5; 95% confidence interval, 1.7 to 63.7; p=0.01). CONCLUSIONS: Trabeculectomy with mitomycin C was generally effective for the treatment of neovascular glaucoma, especially in patients over 50 years of age. However, visual loss in eyes with neovascular glaucoma was common.
Follow-Up Studies ; Glaucoma ; Glaucoma, Neovascular* ; Humans ; Intraocular Pressure ; Mitomycin* ; Risk Factors ; Trabeculectomy*