ERG and OCT findings of a patient with a clinical diagnosis of occult macular dystrophy in a patient of Ashkenazi Jewish descent associated with a novel mutation in the gene encoding RP1L1.

A 57-year-old man with a past medical history of diabetes presented for consultation with a several year history of slowly progressive vision loss in both eyes, which continued to deteriorate over 7 years of follow-up. Multimodal imaging was performed and was significant for the following: on spectral domain optical coherence tomography, a gap lesion was present in the ellipsoid layer, beneath the umbo, as well as subtle macular changes on auto fluorescence imaging. Multifocal electroretinography was performed and was abnormal, and a clinical diagnosis of occult macular dystrophy was made. The patient was subsequently evaluated with genetic testing that revealed a novel p.P73S:c 217C>T nonsense mutation within the retinitis pigmentosa 1-like-1 (RP1L1) gene. The clinical significance of the identified variation will require further investigation.

Anterior Chamber Iris Claw Lens for the Treatment of Aphakia in a Patient with Megalocornea.

Megalocornea in isolation is a rare congenital enlargement of the cornea greater than 13 mm in diameter. Patients with megalocornea are prone to cataract formation, crystalline lens subluxation, zonular deficiencies and dislocation of the posterior chamber intraocular lens (PCIOL) within the capsular bag. A 55-year-old male with megalocornea in isolation developed subluxation of the capsular bag and PCIOL. The PCIOL and capsular bag were explanted, and the patient was subsequently implanted with an anterior chamber iris claw lens. An anterior chamber iris claw lens is an effective option for the correction of aphakia in patients with megalocornea.

Contact lens correction for an aphakic patient with megalocornea.

A 55-year old pseudophakic man with megalocornea presented with a progressive monocular diplopia. On examination the patient was found to have subluxation of the posterior chamber intraocular lens (IOL)/capsular-bag-complex. Surgical removal of the complex was performed resulting in aphakic status. The patient was subsequently fitted with a custom contact lens for aphakia, with successful visual rehabilitation.

Streptococcus agalactiae endogenous endophthalmitis.

Streptococcus agalactiae (SA) is a Group B Streptococcus, which is a common pathogen implicated in neonatal and geriatric sepsis. Endogenous bacterial endophthalmitis (EBE) is a condition that results from haematogenous seeding of the globe, during transient or persistent bacteremia. We document a case of a non-septic geriatric patient, who developed EBE after a transient bacteraemia with SA.

Acute diplopia as the presenting sign of silent sinus syndrome.

Silent sinus syndrome is a rare acquired condition that typically presents as enophthalmos and hypoglobus due to atelectasis of the maxillary sinus. The chronic negative pressure in the sinus slowly retracts the orbital floor, altering orbital anatomy and affecting the function of orbital contents. The authors present the first case of acute vertical diplopia as the presenting symptom of silent sinus syndrome.