Philippines ; Glaucoma syndrome

Marfan syndrome is an uncommon congenital disease originally described by Marfan in 1896. It is inherited as an autosomal dominant trait and can affect the tissues of mesodermal origin, mainly skeletal, ocular, and cardio-vascular systems. We report 4 cases of Marfan syndrome occurring in one family, one of which was found to have an acute attack of glaucoma and underwent extract operation of a right dislocated lens. A brief summary of the literature is submitted.
Glaucoma ; Humans ; Marfan Syndrome ; Mesoderm

Waardenburg syndrome is a very rare auditory-pigment syndrome with autosomal dominant inheritance. In his first report in 1951, Waardenburg characterized this syndrome as dystopia canthorum, a high broad nasal bridge, synophrys[confluent eyebrows], heterochromia iridid, a white forelock or early graying, and sensory neural deafness. We observed a case of Type 2 Waardenburg syndrome with openangle glaucoma and, therefore report with literature review.
Deafness ; Glaucoma ; Glaucoma, Open-Angle* ; Waardenburg Syndrome* ; Wills

Marfan's syndrome is a widespread disturbance of mesodermal tissue throughout the body. The main disturbances are characteristic skeletal anomaly, cardiopathy and eye disturbance such as lens dislocation, glaucoma, etc. Marfan originally described it under the name of dolichostenomelie in 1896. Since then, many cases have been reported in the literature. Authors experienced 3 cases of Marfan's syndrome and 2 cases were operated. The result of surgical treatment. other clinical findings, and brief review of literature are reported as following.
Glaucoma ; Lens Subluxation ; Marfan Syndrome* ; Mesoderm

Essential iris atrophy is a disease characterized by corectopia, iris atrophy, iris hole formation, corneal endothelial degeneration, peripheral anterior synechia, and glaucoma. This disease shares common properties with Chandler's syndrome and Cogan-Reese syndrome, so above three diseases are catagorized by iridocorneal endothelial(ICE) syndrome. We have experienced a patient who had a complaint of visual dimness in right eye. On examination, she had corectopia, iris hole and atrophy, and increased IOP in right eye. Specular microscopic finding was consistent with essential iris atrophy.
Atrophy* ; Glaucoma ; Humans ; Iridocorneal Endothelial Syndrome ; Iris*

Objective: This study investigated ocular complications, such as cataract and glaucoma, arising from prolonged corticosteroid therapy in children. Methods: A cross-sectional study involving pediatric patients with nephrotic syndrome was conducted in a tertiary hospital. Comprehensive ophthalmic assessments including best-corrected visual acuity (BCVA), intraocular pressure (IOP), slitlamp and fundus examination were performed. Standard automated perimetry (SAP) was also performed on patients suspected of having glaucoma. Information on renal histological diagnosis and treatment regimen in each patient was noted. Data were analyzed statistically. Results: A total of 22 patients were evaluated. The median age at the time of examination was 9.5 years (range, 2 to 17 years). The mean age of onset was 6.9 ± 4.3 years. Twelve of the 22 patients had relapses with a mean of 1.86. The mean duration of steroid use was 28 ± 28.9 months. Eleven patients (50%) were given combined therapy (prednisone with either cyclosporine or cyclophosphamide) and 11 were given oral prednisone alone. The mean dose of steroid at the time of examination was 27 ± 26.2 mg/m2 /day. Among the 22 patients, 3 (13.6%) developed posterior subcapsular cataracts. One patient developed steroidinduced glaucoma with a scotoma encroaching the central 10o visual field. There was a significant association between the duration of corticosteroid treatment and cataract formation (p = 0.04), but no significant association between the duration of therapy and development of glaucoma (p = 0.45). Conclusions Cataract formation was a more common complication of prolonged oral corticosteroid therapy with a prevalence rate of 13.6%. Pediatric patients with a longer duration of steroid therapy are at greater risk of cataract formation. Hence, pediatricians are advised to refer these patients to ophthalmologists for proper evaluation.
Cataract Glaucoma Nephritic Syndrome Intraocular Pressure

Human ; Adult ; GLAUCOMA-OPEN-ANGLE ; GLAUCOMA,SIMPLEX ; GLAUCOMA, COMPENSATED ; IRIDOCORNEAL ENDOTHELIAL SYNDROME ; FILTERING SURGERY ; GLAUCOMA ; SCLEROSTOMY ; TRABECULECTOMY

Twenty years old Korean male patient with Hallermann-Streiff syndrome who developed glaucoma is reported. The clinical features are dyscephalia with mandibulo-oculo-facial malformation, bilateral congenital cataract, dental anomalies, hypotrichosis, microphthalmia, nanism and the secondary complications. A brief review of literature is described.
Cataract ; Dwarfism ; Glaucoma ; Hallermann's Syndrome* ; Humans ; Hypotrichosis ; Male ; Microphthalmos

Progressive iris atrophy is a disease characterized by marked iris atrophy, iris hole, corneal endotherial abnormality, perripheral anterior synechia, and glaucoma. It is considered as a variant of iridocorneal endotherial syndrome with Chandler's syndrome and Cogan-Reese syndrome. We have experianced a 34 year old male patient complaning visual blurring in his left eye. At examination, we found characteristics of progressive iris atrophy. After filerring surgery, associated glaucoma was well controlled. Then we report a case with review of the literatures.
Adult ; Atrophy* ; Glaucoma ; Humans ; Iridocorneal Endothelial Syndrome ; Iris* ; Male

PURPOSE: To investigate the factors associated with glaucoma in patients with pseudoexfoliation syndrome by comparing features of the anterior segments and ocular biometry according to the presence or absence of open-angle glaucoma in pseudoexfoliation syndrome. METHODS: We analyzed 96 patients (115 eyes) diagnosed as having pseudoexfoliation syndrome in this study. The patients were divided into two groups of simple pseudoexfoliation syndrome (64 patients, 76 eyes) and pseudoexfoliation glaucoma (32 patients, 39 eyes). We compared the age, sex, underlying disease, location of pseudoexfoliative material, iris change, degree of nuclear cataract, pupil dilatation, corneal endothelial cell counts, central corneal thickness, anterior chamber depth, axial length, corneal curvature, and intraocular pressure (IOP). RESULTS: There were no significant differences between the two groups in terms of age (p = 0.694), sex (p = 0.161), diabetes (p = 0.440), hypertension (p = 0.238), pseudoexfoliative material observed in anterior capsule (p = 0.700), pupillary margin (p = 0.210), iris depigmentation (p = 0.526), pupillary ruff loss (p = 0.708), degree of nuclear cataract (p = 0.617), pupil dilatation (p = 0.526), central corneal thickness (p = 0.097), anterior chamber depth (p = 0.283), axial length (p = 0.095), or horizontal and vertical corneal curvature (p = 0.066 and 0.306, respectively). In pseudoexfoliation glaucoma, significantly higher IOP (p = 0.026), a high frequency of membrane formation (p = 0.047), and decreased corneal endothelial cell counts (p = 0.048) were observed. CONCLUSIONS: Pseudoexfoliation syndrome with open-angle glaucoma was shown to be associated with high IOP, decreased corneal endothelial cell counts, and a high frequency of membrane formation. Therefore, when such changes are observed in pseudoexfoliation syndrome patients, a higher risk of open-angle glaucoma should be recognized, and careful attentionis required accordingly.
Anterior Chamber ; Biometry ; Cataract ; Dilatation ; Endothelial Cells ; Exfoliation Syndrome ; Glaucoma ; Glaucoma, Open-Angle ; Humans ; Hypertension ; Intraocular Pressure ; Iris ; Membranes ; Pupil