Combined Intrastromal Voriconazole and Amphotericin B Treatment for Persistent Fungal Keratitis.

PURPOSE: To evaluate the clinical outcomes of combined intrastromal voriconazole and intrastromal amphotericin B for the treatment of persistent fungal keratitis. METHOD: Patients who received combined corneal intrastromal voriconazole (0.05 mg/0.1 mL) and intrastromal liposomal amphotericin B (0.01 mg/0.1 mL) injections in addition to topical therapy for treatment of persistent fungal keratitis were included in the study. Persistence was described as no improvement or progression in the clinical findings despite treatment with combined topical voriconazole (1 mg/0.1 mL) and topical amphotericin B drops (0.15 mg/0.1 mL) hourly for at least 10 days. The healing of keratitis was considered as the complete closure of epithelial defect with complete resolution of a corneal infiltrate. RESULTS: Thirty-two eyes of 32 patients who met the inclusion criteria were included in this study. Predominant organisms in fungal isolates were Aspergillus species. Combination therapy of intrastromal amphotericin B and intrastromal voriconazole in addition to topical therapy resulted in complete resolution of persistent fungal keratitis in 28 (87.5%) patients. The mean number of intrastromal injections was 9.3±6.4 and ranged from 1 to 18. The mean best-corrected visual acuity values improved from 2.17±0.43 to 1.76±0.77 logarithm of the minimum angle of resolution units (P=0.003). The mean duration of complete epithelial closure was 45.3±16.3 days. Four patients required therapeutic penetrating keratoplasty because of persistence of fungal keratitis (3 patients) and progression of keratitis (1 patient). There was no need for evisceration. CONCLUSION: Combination therapy with intrastromal voriconazole and intrastromal amphotericin B may be an effective adjunct treatment for persistent fungal keratitis.

PARACENTRAL ACUTE MIDDLE MACULOPATHY IN PRIMARY CONGENITAL GLAUCOMA.

PURPOSE: To report a case of primary congenital glaucoma associated with paracentral acute middle maculopathy. METHODS: A case report. RESULTS: A 14-year-old girl with undiagnosed primary congenital glaucoma was referred for the evaluation of sudden vision loss a week after the initial symptoms. Visual acuity was counting fingers at 2 m in the right eye. Examination of her right eye revealed corneal stromal scar like Haab striae, splinter hemorrhage at the temporal border of the optic disk, perivenular hemorrhage, and fern-like white area at the macula. On optical coherence tomography of the right eye, hyperreflective plaques at the level of the inner nuclear layer/outer plexiform layer were present, corresponding to the opaque areas in fundoscopy. On optical coherence tomography angiography of the right eye, ischemic areas in deep capillary plexuses were present. These findings in optical coherence tomography angiography and optical coherence tomography were consistent with paracentral acute middle maculopathy. CONCLUSION: This is the first case of primary congenital glaucoma associated with paracentral acute middle maculopathy in the literature, and primary congenital glaucoma might be a predisposing factor.

Effects of Congenital Ocular Toxoplasmosis on Peripheral Retinal Vascular Development in Premature Infants at Low Risk for Retinopathy of Prematurity

Congenital toxoplasmosis and retinopathy of prematurity (ROP) are two devastating clinical entities of the newborn. There is little information in the literature about the interaction between congenital infections and retinal vascular development at the fetal stage, and none regarding the relationship between ROP and congenital toxoplasmosis. In this report, we present two premature newborns diagnosed with congenital toxoplasmosis with ocular involvement, accompanied by ROP with interrupted retinal vascularization, peripheral avascular regions, and retinal detachment. The aim of this paper is to emphasize the possibility of ROP and congenital toxoplasmosis coexistence wherein one condition may mask the other and make it difficult to distinguish the cause of retinal detachment. Timely management with medical and surgical treatment of congenital toxoplasmosis and ROP could save eyes and vision in those cases.

High incidence of co-existing factors significantly modifying the phenotype in patients with Fabry disease.

Fabry disease results from deficiency of the lysosomal enzyme alpha-galactosidase A. The families of 11 index cases were screened by enzyme and molecular assays. Further clinical and laboratory investigations were carried out in all cases. Including 33 new patients, a total of 28 females (Age 25,82 ±â€¯12,1 Range 8-46) and 16 males (Age 24,56 ±â€¯15,04 Range 2-48) were investigated. Ten different disease-causing variants were found two of them being novel. One patient had co-existing familial mediteranian fever, one had celiac disease and three had rheumatological disorders. Lipoprotein (a) levels were elevated in 17,6%, homocysteine in 22,2%, total and low density cholesterol in 12% and antithrombin 3 levels were elevated in 13,3%. One patient was found to be heterozygous for prothrombin p.G20210A disease-causing variant (5,8%) and two for factor V Leiden disease-causing variant (11,7%). Anticardiolipin IgM antibody was found to be positive in 11,7%. The patients with abnormal cranial imaging were also noticed to have additional risk factors for thrombosis. This study provides the largest data about Fabry patients from Turkey and implies that co-existing risk factors unrelated to Fabry Disease have significant association with the presence of clinical symptoms in females and might cause an early and severe clinical course in males.

The efficacy of orbital ultrasonography and magnetic resonance imaging findings with direct measurement of intracranial pressure in distinguishing papilledema from pseudopapilledema.

INTRODUCTION: The goal of this study was to evaluate the utility of orbital ultrasonography and magnetic resonance imaging in the diagnosis of idiopathic intracranial hypertension (IIH). METHOD: We reviewed the medical records of patients referred to our department for suspected IIH. RESULTS: Seven children were diagnosed with IIH. Nine children revealed pseudopapilledema by optic coherence tomography and/or orbital ultrasonography. When the axial sequences were reexamined, patients with papilledema had optic nerve sheath (ONS) enlargement (6.62 ± 0.70 mm); patients with pseudopapilledema had ONS diameter as 4.62 ± 0.64 mm. There was a significant correlation between the CSF opening pressure and ONS diameter (p < 0.005, r = 0.661). In the papilledema group, the presence of proposed subtle markers as increased tortuosity in the optic nerve was found in six patients. Five of seven patients had a target sign, intraocular protrusion of the optic nerve, and posterior globe flattening. DISCUSSION: Ophthalmological review is important to avoid unnecessary procedures for detection of true papilledema. ONS diameter is a reliable neuroimaging marker as other subtle markers.

A case of postganglionic Horner syndrome after catheterization of internal jugular vein confirmed with pharmacological tests.

Horner syndrome, consisting of ipsilateral miosis, upper eye lid ptosis, and facial anhidrosis, has been reported as a rare complication of internal jugular vein catheterization. In this paper, we describe a nine-year-old girl presenting with postganglionic Horner syndrome, in whom right-sided ptosis and miosis occurred after ipsilateral percutaneous internal jugular venous catheterization.

Axenfeld-Rieger syndrome associated with truncus arteriosus: a case report.

The aim of this presentation was to report a case with Axenfeld-Rieger syndrome (ARS) associated with truncus arteriosus (TA). We present a 14-year-old boy with ARS in whom the diagnosis was confirmed by ophthalmologic examination and developmental defects of the teeth and facial bones. Echocardiography revealed TA. With this case demonstrating the association between ARS and TA, the range of reported cardiac malformations is enlarged and the importance of cardiologic evaluation is emphasized in patients with ARS.