Şansal Gedik

Indocyanine Green and Fundus Fluorescein Angiographic Findings in Patients with Active Ocular Behcet's Disease

Purpose: To define the indocyanine green (ICG) and fundus fluorescein angiographic (FFA) features of Behçets disease. Methods: The study included 49 eyes of 25 patients (19 males, 6 females; mean age: 34.1 years; age range: 14–68 years) with Behçets disease and active ocular involvement that fulfilled the criteria of the International Study Group for Behçets Disease. In each patient, FFA and ICG angiography were performed consecutively in the same session using a TRC-50I/A fundus camera (Topcon, Tokyo, Japan) coupled to an Image Net digitizing system. All images were analyzed and FFA, ICG, and clinical findings summarized. Results: The mean duration of ocular involvement was 52.4 months (range: 2–240 months). FFA showed staining and dye leakage at the optic disc in 44 (89.8%) eyes and diffuse vasculitis in 36 (73.5%) eyes. Macular edema and ischemia were observed in 31 (63.3%) and 3 (6.1%) eyes, respectively. Eight (16.3%) eyes exhibited detectable retinal FFA alterations, but no abnormalities on ICG angiography. Disc ICG hyperfluorescence was observed in 23 (46.9%) eyes. ICG angiography revealed choroidal fuzziness in 16 (32.6%) eyes, hyperfluorescent spots in 13 (26.5%) eyes, and hypofluorescent plaques in 12 (24.5%) eyes. Eleven eyes (22.4%) showed no abnormal findings on ICG angiography. Conclusion: ICG and FFA complement each other as tools for diagnosing patients with Behçets disease. FFA often serves as a fairly reliable guide. Although some ICG findings cannot be demonstrated by FFA, they are not specific or pathognomonic. We do not recommend performing both procedures for Behçets disease.

Congenital cataract, microphthalmia, hypoplasia of corpus callosum and hypogenitalism: Report and review of Micro syndrome

We report on a 7‐month‐old boy with Micro syndrome who was referred for assessment of mental‐motor retardation and reduced vision with cataract. The characteristics of Micro syndrome are mental retardation, microcephaly, congenital cataract, microcornea, microphthalmia, agenesis/hypoplasia of the corpus callosum, and hypogenitalism. The differential diagnosis includes cerebro‐oculo‐facio‐skeletal syndrome (COFS); a syndrome involving cataract, arthrogryposis, microcephaly, and kyphoscoliosis (CAMAK); a syndrome with cataract, microcephaly, failure to thrive, and kyphoscoliosis (CAMFAK); Martsolf syndrome; Neu–Laxova syndrome; Lenz microphthalmia syndrome; and Smith–Lemli–Opitz syndrome. Till date, no renal malformations have been reported in Micro syndrome. Our patient had fusion of the lower poles of the kidneys and his left kidney was ectopic. Ocular findings are the most reliable neonatal diagnostic signs of Micro syndrome. Minör anomalies in Micro syndrome may be subtle and therefore not of significant diagnostic value. Micro syndrome is an autosomal recessive trait. Till date, most reported cases have been in individuals of Muslim origin. In countries with high rates of consanguineous marriage, such as Turkey, it is important that physicians be able to recognize this syndrome. Micro syndrome should be considered in any infant with congenital cataract.

Stroke in Early Childhood Due to Homocystinuria

A previously healthy girl, age 3 years 9 months, presented with right-sided hemiparesis and seizures. Ischemic infarction was confirmed through magnetic resonance imaging and magnetic resonance angiography. Extensive evaluation to discover the underlying etiologies and risk factors predisposing this patient to stroke included coagulation defects, cardiac anomalies, congenital inborn metabolism deficiency, and infections and trauma. Based on the clinical and laboratory results, a diagnosis of homocystinuria was made. Homocystinuria is an inherited disorder that affects the metabolism of the amino acid methionine. Although homocystinuria is usually associated with ischemic strokes, the sudden onset of stroke as the initial clinical presentation of homocystinuria is very rare in early childhood. Based on this case, however, metabolic screening for hyperhomocystinemia is recommended in any child presenting with a stroke.

Characteristics of the Anterior Segment Biometry and Corneal Endothelium in Eyes with Pseudoexfoliation Syndrome and Senile Cataract.

Objectives: To evaluate the anterior segment biometric features and corneal endothelial changes in eyes with pseudoexfoliation (PEX) syndrome and senile cataract. Materials and Methods: The central corneal thickness, anterior chamber depth (ACD), pupil diameter, lens thickness, endothelial cell density (ECD), and percentages of polymegathism and pleomorphism of 52 subjects with PEX and cataract were compared with 51 age- and gender-matched control subjects with cataract using optical low-coherence reflectometry (OLCR, Lenstar LS 900; Haag Streit AG, Switzerland) and in-vivo confocal microscopy (Confo Scan 4, Nidek Co. Ltd, Osaka, Japan). Nineteen subjects with PEX syndrome had glaucoma and were using anti-glaucoma medications. Only one eye of the subjects was used in statistical analysis and a p value less than 0.05 was considered statistically significant. Results: None of the OLCR parameters reached statistically significant differences among the 3 groups (ANOVA p>0.05). The percentage of eyes with ACD <2.5 mm was 13.7% in the control group, 24.2% in PEX eyes without glaucoma and 21.1% in PEX eyes with glaucoma, with no statistically significant differences (p=0.45). There was a significant difference in mean ECD among the 3 groups (ANOVA p=0.02), whereas no differences could be found in respect to polymegathism and pleomorphism (p>0.05). Mean ECD was significantly lower in the PEX glaucoma group (2,199.5±176.8 cells/mm2) than the control group (2,363±229.3 cells/mm2) (p=0.02), whereas no difference was found in mean ECD of PEX eyes without glaucoma and the control group (p=0.42). ECD was less than 2,000 cells/mm2 in 15.8% of PEX subjects with glaucoma, 9.8% of control subjects and 6.1% of PEX eyes without glaucoma, with no statistically significant difference (p=0.52). Conclusion: As eyes with both PEX glaucoma and cataract seem to be associated with decreased endothelial cell number, specular or confocal microscopy screening should be done for the patients scheduled for intraocular surgery.

Bilateral Papillophlebitis in a Patient with Mutation of Metilenetetrahydrofolate Reductase Enzyme

Papillophlebitis is known as central retinal vein occlusion seen in young patients. It usually presents as unilateral optic disc edema with cotton wool spots and hemorrhage in the peripapillary region. As it may be due to many autoimmune and inflammatory causes, a thorough systemic evaluation of the patient is warranted. In this case report we describe a bilateral, simultaneous papillophlebitis case thought to be related to hyperhomocysteinemia secondary to C677T polymorphism of methylenetetrahyrofolate reductase enzyme.

Eyelid Molluscum Contagiosum Lesions in Two Patients with Unilateral Chronic Conjunctivitis

Molluscum contagiosum (MC) is a viral infection of the skin and mucosal tissues characterized by skin-colored or transparent round nodules with a dimple or pit in the center. The infection is caused by a DNA poxvirus called the MC virus. Although MC generally occurs in children, it has also been reported in immunocompromised and atopic patients. The virus is transmitted by skin contact or sexual intercourse. The lesions disappear spontaneously within several months in most cases. However, excision, cryotherapy, cauterization, topical chemical and antiviral agents, and/or oral cimetidine are used in refractory cases or to accelerate the healing process. Herein, we discussed the clinical findings and our treatment of two patients with unilateral chronic conjunctivitis associated with eyelid MC lesions in light of the literature.