TRANSCLERAL DRAINAGE OF SUBRETINAL FLUID, ANTI-VASCULAR ENDOTHELIAL GROWTH FACTOR, AND WIDE-FIELD IMAGING-GUIDED LASER IN COATS EXUDATIVE RETINAL DETACHMENT.

PURPOSE: To evaluate the safety and efficacy of combined transscleral drainage of subretinal fluid (SRF) with intravitreal bevacizumab and laser photocoagulation in the management of advanced Coats disease (Stage 3) with exudative retinal detachment. DESIGN: Retrospective interventional case series. METHODS: Retrospective case review of eight eyes in eight children with advanced Coats disease manifested as total or subtotal retinal detachment. All eyes initially underwent surgical drainage of exudative SRF followed by intravitreal injection of bevacizumab and laser photocoagulation. Patients were subsequently followed up for up to 60 months. RESULTS: In all eyes, after SRF drainage and administration of one to two intravitreal injections, SRF was completely eliminated. Patients required up to four sessions of laser photocoagulation. Retinal detachment consequently reduced with all patients showing total retinal reattachment and resolution of the subretinal exudates. At the last follow-up, no patient showed recurrent SRF and no ocular complications related to bevacizumab nor evidence of further disease progression were noted. CONCLUSION: The authors present a new therapeutic approach that allows for the first time successful treatment of advanced cases of exudative retinal detachment in Coats disease without the need for vitrectomy. Transscleral drainage of SRF accompanied by anti-vascular endothelial growth factor injection and laser photocoagulation appears to be successful in halting progression of advanced Coats disease with exudative detachment and a less invasive approach when compared with conventional management.

Visual outcomes of pars plana vitrectomy with epiretinal membrane peel in patients with uveitis.

PURPOSE: To report the outcomes of pars plana vitrectomy with epiretinal membrane (ERM) peel, with or without internal limiting membrane peel, in patients with uveitis. METHODS: Retrospective interventional case series of patients undergoing pars plana vitrectomy with ERM peel between January 2005 and March 2012. Sixteen consecutive patients (16 eyes) were identified, with a minimum postoperative follow-up of 6 months. Visual acuity, anatomical outcomes, perioperative control of inflammation, and complications were assessed. RESULTS: The mean age at surgery was 47.3 years (range, 14-68 years), with a mean duration of ERM at surgery of 21.3 months (3-84 months). At 6 months, visual acuity improved in 31.25% of eyes, stabilized in 31.25%, and was worse in 37.5%. The causes of reduced visual acuity postoperatively included severe preexisting macular pathology and unoperated cataract. CONCLUSION: Pars plana vitrectomy with ERM peel in eyes with uveitis may improve or stabilize visual acuity, especially in eyes with macular traction, but in the absence of traction, outcomes are variable and unpredictable. Prevention of ERM formation by aggressive control of inflammation is important.

Nephropathic cystinosis presenting with uveitis: Report of a "Can't See, Can't Pee" situation.

Nephropathic cystinosis is a rare autosomal recessive lysosomal disease characterized by accumulation of pathognomonic cystine crystals in renal and other tissues of the body. Cystinosis is caused by mutant cystinosin, the cystine transport protein located in lysosomal membranes, leading to systemic deposits of cystine and resultant end organ damage. Cystinosis is rarer in Asians than Caucasians with only a handful of cases reported from India to date. Due to its extreme rarity and clinically insidious presentation in contrast to the infantile form, the diagnosis of juvenile nephropathic cystinosis is frequently delayed or overlooked. Moreover, routine processing and sectioning of paraffin embedded tissues dissolves cystine crystals, making it difficult to diagnose this condition on light microscopic examination alone, mandating electron microscopic (EM) analysis of renal biopsies for an accurate diagnosis of this condition. We describe a case of juvenile nephropathic cystinosis presenting with uveitis and photophobia in a 17-year-old Indian male, diagnosed after EM examination of the patient's renal biopsy for evaluation of nephrotic syndrome. While highlighting the diagnostic utility of EM, we describe a few histopathologic clues which can prompt inclusion of EM analysis of renal biopsies in this setting.

Twin Reversed Arterial Perfusion Syndrome (TRAP or Acardiac Twin)-A Case Report.

Twin reversed arterial perfusion (TRAP) syndrome is a rare condition (0.3:10,000 births) that occurs in monochorionic twin pregnancies, resulting in coexistence of a normal "pump" twin and an acardiac twin. The acardiac twin is dependent upon the normal twin to provide circulation by means of vascular anastomosis. Many of the bizarre defects are felt to be caused by low oxygen tension and this causes dramatic alteration in the twin fetal physiology and high prenatal mortality. The acardiac twin is a parasite, putting the pump fetus at risk of high output cardiac failure. Overall only 50% of pump twins survive. We present a case in a twenty year female, gravida two, para one, living one, full term normal pregnancy, ultrasound showed twin pregnancy. Following delivery, the first fetus survived only for one day and the second fetus showed features of TRAP syndrome.

Congenital malformations in perinatal autopsy: a two-year prospective study.

Perinatal autopsy detects the cause of death and also finds the various types of congenital malformation involving one or more than one system. Congenital malformations are one of the leading causes of perinatal deaths and infant mortality. In the present study various visceral malformations were detected in perinatal autopsies and categorised them systemwise. The prospective study was conducted during the period of June 2007 to May 2009 consisting of 32 cases. Out of 32 perinatal deaths studied, 30 were stillborn and in 2 there were early neonatal deaths. In each case, an attempt was made to find out the congenital malformation in perinatal deaths and clinicopathological correlation was attempted after a detailed postmortem and histopathological study. Congenital malformations were seen in 10 cases which accounted for 31.2% of perinatal deaths. A total of 64 congenital malformations were observed in 10 cases. Malformations of the alimentary system (20.31%) were most common followed by genito-urinary system (18.75%), musculoskeletal system (17.18%) and central nervous system (9.37%). There were other 22 congenital anomalies. Two cases of harlequin icthyosis and one case each of Meckel Gruber syndrome, sirenomelia and twin reverse arterial perfusion syndrome were also seen. In many of the perinatal deaths, internal malformations were not suspected clinically. Thus, autopsy is an invaluable tool for detecting visceral malformations, adding to the clinical diagnosis and counselling the parents for subsequent pregnancy.