A Case Series of Hereditary Congenital Cataract

@#Congenital cataract is a major cause of preventable blindness in children. It can be either hereditary or non-hereditary. In this case series, we present three cases of congenital cataract with a strong family history. The grandparents and parents of these patients had cataract diagnosed in late childhood whilst the patients in this case series were diagnosed with congenital cataract earlier. There was no history of consanguineous marriage in any of the families. These cases show that, in the presence of a strong family history, a child will present with congenital cataract earlier in their life. Hence, babies from such families should be screened at birth and at regular intervals within the first year of life to enable early diagnosis and subsequent surgical intervention to reduce the incidence and burden of amblyopia.

Ocular Permeation of Topical Tazocin and Its Effectiveness in the Treatment of Pseudomonas aeruginosa Induced Keratitis in Rabbits.

PURPOSE: Pseudomonas aeruginosa is the most common causative organism for contact lens-associated corneal ulcer and is commonly treated with fluoroquinolones. With the emergence of resistant strains, it is important to investigate alternative therapies. Despite well-established efficacy of tazocin against systemic Pseudomonas infections, its topical use for the treatment of Pseudomonas keratitis has not been described, hence this study was aimed to find the ocular permeation of Tazocin and its efficacy in treating keratitis in rabbit eyes. METHODS: We investigated the ocular permeation of topical tazocin after single drop application in normal rabbit eyes by estimating piperacillin and tazobactam concentrations in cornea, aqueous, and vitreous using a validated LC-MS/MS method. Furthermore, we determined the efficacy of repeated dose administration of tazocin against experimentally induced P. aeruginosa keratitis in rabbits in comparison to moxifloxacin. To determine the efficacy, clinical examination, histopathological examination, and estimation of bacterial load and inflammatory cytokines in cornea were done. RESULTS: Significant corneal concentration of piperacillin and tazobactam was detected in normal rabbit corneas after single dose treatment with tazocin. In rabbits with Pseudomonas-induced keratitis, topical tazocin caused significant clinical and histopathological improvement. This improvement was associated with reduction in corneal bacterial load and inflammatory cytokines. Compared to moxifloxacin 0.5%, tazocin treated group showed greater clinical response which was associated with higher interleukin (IL)-1ß, lower tumor necrosis factor (TNF)-α, a comparable level of IL-8, greater reduction in corneal bacterial load, and lesser inflammatory cell infiltration. CONCLUSION: Tazocin showed good ocular penetration and was effective in treatment of Pseudomonas induced keratitis in rabbits.

Neonatal herpes simplex virus type-1 central nervous system disease with acute retinal necrosis.

We report a case of neonatal herpes simplex virus (HSV)-1 central nervous system disease with bilateral acute retinal necrosis (ARN). An infant was presented at 17 days of age with focal seizures. Cerebrospinal fluid polymerase chain reaction was positive for HSV-1 and brain magnetic resonance imaging showed cerebritis. While receiving intravenous acyclovir therapy, the infant developed ARN with vitreous fluid polymerase chain reaction positive for HSV-1 necessitating intravitreal foscarnet therapy. This is the first reported neonatal ARN secondary to HSV-1 and the first ARN case presenting without external ocular or cutaneous signs. Our report highlights that infants with neonatal HSV central nervous system disease should undergo a thorough ophthalmological evaluation to facilitate prompt diagnosis and immediate treatment of this rapidly progressive sight-threatening disease.

Risk factors for clinically significant macular edema in a multi-ethnics population with type 2 diabetes.

AIM: To determine the risk factors of clinically significant macular edema (CSME) in patients with non-proliferative diabetic retinopathy (NPDR) in a multi-ethnics Malaysian population. METHODS: We performed a case control study in which 150 patients with bilateral NPDR and CSME in either eye were compared to 150 patients with bilateral NPDR and no CSME in both eyes. CSME and NPDR were graded according to Early Treatment of Diabetic Retinopathy Study criteria. Student's t-test, odds ratio and multiple logistic regression analysis were performed to analyze the duration of diabetes, body mass index (BMI), blood pressure(BP), total cholesterol, low density lipoprotein (LDL), high density lipoprotein (HDL), triglycerides (TG), fasting blood glucose (FBG), HbA1c, full blood count, serum creatinine and proteinuria between the two groups. RESULTS: Both groups were matched in terms of age, gender and ethnicity. Duration of diabetes, total serum cholesterol, serum LDL, FBG, HbA1c and serum creatinine were significantly higher in the CSME group (P<0.05). The hemoglobin, packed cell volume were significantly lower in the CSME group (p<0.05). There was no significant difference for serum HDL, TG, BMI, systolic and diastolic BP. Multiple logistic regression analysis showed that total serum cholesterol and HbA1c had significantly high odds of developing CSME. CONCLUSION: HbA1c and total cholesterol are the two most important risk factors associated with CSME in patients with NPDR.

Spontaneous dislocation of intraocular lens as a late complication of uncomplicated cataract surgery: a case series.

Five patients having uncomplicated phacoemulsification were implanted with CT-Asphina 603P intraocular lenses into the capsular bag. After a few months, three of the patients had haptic flexion anterior to the optic despite minimal capsular fibrosis. One patient had spontaneous dislocation of the intraocular lens into the anterior chamber with only minimal capsular fibrosis. Another patient had one haptic dislocated out of the bag. Late intraocular lens dislocation is also seen in newly designed preloaded intraocular lenses despite the absence of significant capsular fibrosis. Haptic-optic junction design and intraocular lens material play important roles in such complication. A slightly large capsulorhexis might be a deterrent for implantation of this intraocular lens.

Topical piperacillin/tazobactam for recalcitrant pseudomonas aeruginosa keratitis.

PURPOSE: To report the treatment of therapy-resistant Pseudomonas aeruginosa keratitis with topical piperacillin/tazobactam. METHODS: Retrospective report of 3 cases. RESULTS: Three patients with P. aeruginosa keratitis were unresponsive to various antimicrobials. Resolution of all 3 cases of keratitis occurred upon commencement of topical piperacillin/tazobactam. CONCLUSION: Therapy-resistant P. aeruginosa keratitis acquired from the community is becoming an increasing problem. Topical piperacillin/tazobactam is an option for the treatment of therapy-resistant P. aeruginosa keratitis.

47 XYY and morning glory syndrome--a unique association.

47 XYY syndrome is a sporadic condition in which the human male receives an extra Y chromosome. Few ocular associations have been documented. The authors report the first case of 47 XYY associated with morning glory syndrome, frontonasal meningoencephalocele, and midfacial defects.

Presumed spontaneously regressed retinoblastoma with tractional retinal detachment and dislocated lens / 国际眼科杂志(Guoji Yanke Zazhi)

We illustrated a case of presumed spontaneously regressed retinoblastoma in a blind eye. The tumour remained stable, with no extraocular spreading, for 20 years. Associating signs included band keratopathy, leukocoria, dislocated lens, calcified retinal masses and tractional retinal detachment.