Ectopic vortex veins and varices in Donnai Barrow syndrome.

BACKGROUND: Donnai Barrow Syndrome (DBS) is a rare, multi-system autosomal recessively inherited disorder of relevance to ophthalmologists. To aim to describe the ocular phenotype using multimodal imaging for two cases of genetically confirmed DBS and compare against the published phenotype. MATERIALS AND METHODS: Retrospective case series of two unrelated patients with DBS and review of the literature. Both cases were referred to our tertiary unit for laser prophylaxis against retinal detachment. RESULTS: There was extreme high myopia greater than 20 dioptres without rhegmatogenous retinal detachment (RRD). Anterior segment features included iris transillumination and ciliary body hypoplasia. Posterior segment changes included previously described changes of optic nerve hypoplasia and a strikingly abnormal appearance of the fundus consisting of multiple bilateral giant posterior vortex veins (PVV). The mouse model shows a similar phenotype. CONCLUSIONS: Ectopic vortex veins of the choroid expand the phenotype of DBS and can be helpful in distinguishing the differential diagnosis of high myopia in children. Posterior vortex veins have been described in adult high myopia as acquired but our cases suggest that they could be congenital. Orbital manipulation and hypotony during surgery should be avoided to minimise complications. The evidence to recommend prophylactic laser retinopexy in these cases is inconclusive, and overall we recommend that conservative management should be considered using wide-angle retinal imaging in the clinic.

Evolving outcomes of surgery for retinal detachment in retinopathy of prematurity: the need for a national service in the United Kingdom : An audit of surgery for acute tractional retinal detachment complicating ROP in the UK.

OBJECTIVES: To audit the structural and functional outcomes of surgery for acute tractional retinal detachment due to retinopathy or prematurity between 2004 and 2014 in Oxford UK. METHODS: Consecutive operations were identified from a surgical log. Clinical data including demography, perioperative data, and retinal outcomes were extracted into a spreadsheet and compared against two international data sets referenced in the method section. Nonparametric tests (Fisher's exact, and the Mann-Whitney U-tests) were used for statistical analysis with a p-value < 0.05 considered significant. RESULTS: Twenty-nine eyes of 19 babies underwent surgery. The mean age (SD) at final follow-up was 6.4 (3.7) years of age and comparable to the reference data sets. The mean birth weight and gestational age of babies matched the ETROP data set referenced in the method section. Anatomical success was obtained in 16/29 (55.2%) of eyes and more likely with stage 4 ROP than stage 5 disease (p < 0.05). Thirteen of 29 eyes (44.8%) obtained form vision post-operatively. All instances of macular retinal reattachment during follow up were verified with post-operative OCT. CONCLUSIONS: Surgery for stage 5 ROP is not worthwhile. For stage 4 ROP it yielded better visual outcomes than ETROP but registration for visual impairment was not prevented. Innovation such as endoscopic vitrectomy could yield better outcomes. Earlier detection of vitreoretinal fibrosis could result in timelier referral. A formally funded national service is needed to ring-fence resource to avoid delays in access to surgery, which has a narrow surgical window.

Binocular Indirect Ophthalmoscopy Complements Non-contact Wide-field Imaging with Optos to Treat a Baby Outside ETROP Guidelines.

We report a male premature baby who was born at 24 weeks gestation weighing 600 grams. There was bilateral stage 2, zone 2 retinopathy of prematurity (ROP) without plus disease at 38 weeks postmenstrual age. Ultra-wide-field Optomap images obtained 1 week later showed no change in ROP stage. At 40 weeks postmenstrual age, stage 3, zone 2 ROP was detected using binocular indirect ophthalmoscopy and documented using Optos. Minor tortuosity and dilation of vessels was interpreted as pre-plus disease. One week later, at 41 weeks postmenstrual age, Optomap images identified progressive extraretinal fibroproliferation in the nasal quadrant. As a result, the baby was treated with fundus laser photocoagulation.

Ultra-Widefield Imaging for Pediatric Retinal Disease.

This article explores the development of retinal imaging, with particular emphasis on ultra-widefield imaging and the key concept of field of view. Two ultra-widefield imaging platforms are examined in detail-Optomap and Spectralis-noncontact imaging systems that include protocols for performing angiography in infants. Applications of ultra-widefield imaging are illustrated using case studies, including diagnosis, monitoring, and screening.

Unilateral persistent hyperplastic primary vitreous: intensive management approach with excellent outcome beyond visual maturation.

Persistent hyperplastic primary vitreous (PHPV) is an ocular developmental disorder resulting from incomplete apoptosis of the embryonic hyaloid vasculature. Unilateral PHPV is traditionally associated with a poor prognosis because of the challenges associated with managing progressive anisometropic amblyopia. We report a child with unilateral PHPV who underwent cataract extraction, primary posterior capsulotomy with anterior vitrectomy and intraocular lens implantation followed by combined trabeculectomy/trabeculotomy within the first 8 weeks of life. Intensive optometric and orthoptic input was required for many years to manage the increasing anisometropic amblyopia with final visual acuity of 20/40 unaided in the affected eye and without evidence of glaucomatous optic neuropathy. This case illustrates the excellent visual outcome possible in a child with complex, unilateral PHPV using an intensive management approach comprising: early surgical intervention for congenital cataract and secondary glaucoma, meticulous monitoring of refraction, visual acuity and intraocular pressure and motivated parents who engaged in the management.

Silicone oil pupil block glaucoma in a pseudophakic eye.

Intravitreal silicone oil achieves an effective endotamponade in patients with complex retinal detachments. Silicone oil displacement into the anterior chamber risks glaucoma and endothelial failure. We describe a 52-year-old patient with pseudophakia with silicone oil endotamponade presenting with visual loss and intraocular pressure of 60 mm Hg. Inferior YAG iridotomy was undertaken to repatriate silicone oil to the posterior segment. Despite normal intraocular pressure, acute corneal oedema occurred postiridotomy, resolving spontaneously over 2 weeks. Pupil block glaucoma secondary to silicone oil requires a management approach based on an understanding of silicone oil fluidics. Careful selection of inferior laser iridotomy site is critical to effectively reverse pupil block. Anterior migration of silicone oil in patients with pseudophakia is rare. We offer an hypothesis to explain unanticipated transient corneal oedema following silicone oil displacement from the anterior chamber. Clinicians must discuss the possibility of transient or permanent endothelial failure preoperatively in this patient group.

Anterior sub-Tenon's anesthesia for the treatment of retinopathy of prematurity.

The authors describe a technique of combined oral chloral hydrate and sub-Tenon's anesthesia that is simple, avoids the risks of general anesthesia, and provides superior anesthesia to topical agents alone.

Vitrectomy without postoperative posturing for idiopathic macular holes.

PURPOSE: To determine the success of vitrectomy with ILM peeling and C(3)F(8) tamponade for macular holes without the need for postoperative face-down posturing. METHODS: Twenty-four eyes of 24 consecutive patients undergoing pars plana vitrectomy with indocyanine green-assisted ILM peeling and C(3)F(8) tamponade without prone posturing were included in the study. All patients had follow up on 1 day, 2 weeks and 3 months postoperatively. Biomicroscopy and optical coherence tomography were used to assess macular hole closure at 3 months postoperatively. Snellen visual acuity was compared pre- and postoperatively. RESULTS: Of the 24 eyes recruited, two (8%) had stage II, 17 (71%) had stage III and five (21%) had stage IV macular holes. Nineteen (79%) eyes were phakic and five (21%) eyes were pseudophakic at the time of surgery. The macular holes had been present for an average of 7.5 months (range 3-18 months). At 3-month follow up, 22/24 (91.6%) holes were closed. Both of the two holes that failed to close were stage IV macular holes. Preoperative visual acuity ranged from 6/18 to 6/60 (mean 6/36). Postoperative visual acuity ranged from 6/9 to 6/60 (mean 6/18). Eighteen eyes had improvement of visual acuity of at least one line on the Snellen chart, six eyes had no improvement. No eyes had worse vision postoperatively. CONCLUSION: Macular hole surgery without face-down posturing provides anatomical and functional results comparable to those with prone posturing. Combined phacovitrectomy is not essential to avoid prone posturing.

Repair of macular fold following retinal reattachment surgery.

Here the management of a macular fold complicating retinal reattachment surgery is discussed. The macular fold was repaired by a technique similar to that used in retinal translocation surgery. Direct injection into the macular fold was performed, which caused a linear, rather than the intended concentric, detachment of the retina. Permanent flattening of the macula fold was achieved and the best-corrected Snellen visual acuity was 6/12 at 6 months. The importance of intervention, advantages and disadvantages of this technique and alternative methods of managing such cases are discussed. It may be prudent to consider peripheral, rather than direct, injection into the macular fold to detach the macula in such cases.

Optical coherence tomography in the management of acute retinopathy of prematurity.

PURPOSE: To demonstrate the microscopic changes, in vivo, accompanying stage 4 retinopathy of prematurity. DESIGN: Interventional case study. METHODS: Optical coherence tomography (OCT) of the macula was performed in a baby with presumed stage 4a retinopathy of prematurity (retinopathy of prematurity). The findings were correlated with clinical staging and video indirect ophthalmoscopy. RESULTS: OCT changed the staging of retinopathy of prematurity from 4a to 4b, demonstrated intraretinal edema, and objectively confirmed the clinical impression of disease progression. CONCLUSIONS: OCT could be valuable as a new strategy in the staging and surgical management of retinopathy of prematurity.

Posterior chamber compartments demonstrated by optical coherence tomography, in silicone-filled eyes, following macular hole surgery.

PURPOSE: To report visualization with optical coherence tomography of a prefoveal space in silicone oil-filled eyes following surgery for idiopathic macular hole. METHODS: Seven eyes of seven consecutive patients undergoing macular hole surgery with silicone oil tamponade were prospectively evaluated with optical coherence tomography preoperatively, on the first postoperative day, after 6 weeks and before removal of silicone oil. RESULTS: In all seven eyes the posterior face of the silicone oil bubble was visualized with optical coherence tomography. In all of these patients, who had a silicone oil fill of over 90%, the silicone oil bubble in the upright position did not conform to the foveal depression. Closure of the macular hole was achieved in all seven eyes after one operation. CONCLUSIONS: Macular hole closure can occur without complete tamponade of the central fovea. A prefoveal space can be visualized in silicone oil-filled eyes. Concentration of growth factors, adjuvants and indocyanine green in this compartment may influence postoperative visual outcome, as may changes in retinal homeostasis induced by removal of silicone oil.