Enhanced S-cone syndrome: Clinical spectrum in Indian population

Purpose: Enhanced S-cone syndrome (ESCS), a rare disorder, is often misdiagnosed as other forms of retinal degenerations, which have a poorer prognosis than ESCS. The aim of this study is to report the varied clinical features of ESCS and distinguish it from other similar disorders. Methods: We retrospectively scrutinized the records of patients with confirmed diagnosis of ESCS and analyzed the findings. Results: We included 14 patients (age range 4–39 years) who were confirmed to have ESCS according to pathognomonic electroretinography (ERG) showing reduced photopic, combined responses, and 30 Hz flicker with reduced L, M cone responses and supernormal S cone responses. The disease presented in the 1st decade with night blindness and was almost stationary or minimally progressive. Mid-peripheral fundus changes in form of nummular pigmentary alterations, yellow punctate lesions, and macular schisis were noted. The vision ranged from 6/6 to 6/36 with follow-up ranging from 1month to 22 years. Conclusion: ESCS shows varied clinical features ranging from unremarkable fundus to pigment clumping and atrophic lesions. It has good prognosis with patients mostly maintaining their vision. ERG is diagnostic. More awareness and knowledge about this entity can help to differentiate it from other forms of night blindness.

Outcome of vitreoretinal surgery for rhegmatogenous retinal detachment in X-linked juvenile retinoschisis

Purpose: Rhegmatogenous retinal detachment (RRD) is a vision-threatening complication of X-linked juvenile retinoschisis (XLRS). The aim of this study is to report the anatomical and functional outcomes of vitreoretinal surgery for the treatment of RRD in X-linked juvenile retinoschisis (XLRS). Methods: This is a retrospective, interventional, consecutive case series in which case records of 34 eyes of 28 XLRS patients, who underwent surgery for RRD, were reviewed. Statistical analysis used is as follows: visual outcomes were categorized into three groups: improved, remained stable or deteriorated. Wilcoxon signed-rank test was used to determine the difference in visual acuity at baseline and at final visit. Univariate binary logistic regression analysis was used to determine the risk factors for retinal detachment. Any P value < 0.05 was considered as statistical significant. Results: Mean age of the boys at presentation was 9.2 ± 3.5 years. Indication for surgery was RRD in all eyes. Concurrent vitreous haemorrhage was present in four eyes (11.8%). The primary surgical intervention was scleral buckle in 12 eyes and pars plana vitrectomy in 22 eyes. Persistence of subretinal fluid/redetachment was seen in 15 eyes of which 11 eyes underwent additional surgeries. At final follow up, 27 eyes (79.4%) were noted to have an attached retina. Of the total eyes, in 20, 6 and 8 eyes the visual acuity improved, stabilized and deteriorated, respectively. Conclusion: Favourable outcomes are seen in a majority of eyes after vitreoretinal surgery for RRD in XLRS; however, multiple surgical interventions may be necessary.

Polypoidal choroidal vasculopathy: Pearls in diagnosis and management

Polypoidal choroidal vasculopathy (PCV) is increasingly recognized as an important cause of exudative maculopathy in Asians as against Wet age-related macular degeneration in Caucasians. A panel of retinal experts methodically evaluated pertinent updated literature on PCV with thorough PubMed/MEDLINE search. Based on this, the panel agreed upon and proposed the current consensus recommendations in the diagnosis (clinical and imaging), management and follow-up schedule of PCV. Diagnosis of PCV should be based on the gold standard indocyanine green angiography which demonstrates early nodular hyperfluorescence signifying the polyp with additional features such as abnormal vascular network (AVN). Optical coherence tomography is an excellent adjuvant for diagnosing PCV, monitoring disease activity, and decision-making regarding the treatment. Current treatment modalities for PCV include photodynamic therapy, anti-vascular endothelial growth factor agents, and thermal laser. Choice of specific treatment modality and prognosis depends on multiple factors such as the location and size of PCV lesion, presence or absence of polyp with residual AVN, amount of submacular hemorrhage, presence or absence of leakage on fundus fluorescein angiography, visual acuity, and so on. Current recommendations would be invaluable for the treating physician in diagnosing PCV and in formulating the best possible individualized treatment strategy for optimal outcomes in PCV management.

Optical coherence tomography: A guide to interpretation of common macular diseases

Optical coherence tomography is a quick, non invasive and reproducible imaging tool for macular lesions and has become an essential part of retina practice. This review address the common protocols for imaging the macula, basics of image interpretation, features of common macular disorders with clues to differentiate mimickers and an introduction to choroidal imaging . It includes case examples and also a practical algorithm for interpretation.

End-tidal carbon dioxide monitoring in pediatrics - clinical applications.

End-tidal CO2 monitoring is an exciting non-invasive technology that is more commonly used in the emergency department, intensive care unit and in the prehospital setting. Its main use has been in verifying endotracheal tube position, during mechanical ventilation and cardiopulmonary resuscitation, but it is being studied and used for other purposes as well. The new American Heart Association guidelines require secondary confirmation of proper tube placement in all patients by exhaled CO2 immediately after intubation and during transport. This article covers the clinical applications of end-tidal CO2 monitoring with special reference to the paediatric patient.

Retinal pigment epithelial tears associated with idiopathic central serous chorioretinopathy.

Two patients with idiopathic central serous chorioretinopathy with retinal pigment epithelial tears are described.

Ultrasound Biomiscroscopy in the diagnosis and management of cyclodialysis clefts.

PURPOSE: To report the role of Ultrasound Biomicroscopy (UBM) as a tool in the diagnosis and management of cyclodialysis clefts. METHODS: Six eyes of 6 patients with hypotony and suspected or diagnosed cyclodialysis clefts underwent UBM evaluation. Post-treatment UBM was performed in four eyes to assess the effect of the treatment. RESULTS: Cyclodialysis clefts were accurately diagnosed and delineated in 6 eyes by UBM. Complete closure was documented after treatment in 3 eyes, and a residual cleft in one eye. These findings were compared to gonioscopic findings. CONCLUSIONS: UBM is a safe, accurate and noninvasive diagnostic tool in the diagnosis of cyclodialysis clefts and is of particular use when other conventional methods of diagnosis are inconclusive.

Current concepts in the management of adult intraocular tumours.

The management of intraocular tumours has come a long way since the days when enucleation was the only modality of treatment available to the ophthalmologist. Despite the fact that enucleation is still necessary in certain situations, the emphasis is currently on conservative management, thereby saving the eye and some amount of useful vision wherever possible. This review highlights the current trends in the management of adult intraocular tumours with emphasis on newer diagnostic modalities such as computed tomography, magnetic resonance imaging, and fine needle aspiration biopsy. Only those tumours that are most likely to be seen in clinical practice are included.