Stickler Syndrome (SS) is a significant cause of retinal blindness in children. The immediate cause of blindness is retina detachment from giant retinal tear (GRT). It is frequently diagnosed late and the giant retinal tear (GRT) may be complicated by high-grade proliferative vitreoretinopathy (PVR). The surgery for the combined GRT with PVR has limited structural results and the vision mainly remains impaired. In order to improve the visual outcomes, we propose an organized program oriented toward early diagnosis and surveillance. Adding an effective prophylaxis may maintain normal vision in a high percent of patients. The critical diagnostic moments for this program are prenatal and at birth. The tools include a directed history, general physical exam and advanced ophthalmologic exam looking for the particular features of SS. Some features may need advanced skills transfer, because they are not reliably taught in retina fellowships. Much of this program requires a partnership with obstetricians, pediatricians, neonatologists and geneticists. Finally, we review the evidence regarding prophylaxis and discuss our approach in the absence of guidance from a randomized clinical trial.

Purpose To investigate the postnatal growth and neurodevelopment of infants with retinopathy of prematurity (ROP) treated with intravitreal bevacizumab (IVB). Patients and Methods This was a retrospective comparative study. A total of 262 infants were divided among three study groups: 22 treated with intravitreal bevacizumab, 55 treated with laser, and 185 with ROP that resolved without treatment. Infants with nonviable course or hydrocephalus, a source of non-physiologic weight gain, were excluded. Neurodevelopment was assessed with Bayley III scores at 17–28 months if available and presence of hearing loss or cerebral palsy. Weekly weight, height, and head circumference from birth through 50 weeks postmenstrual age (PMA) were modeled to determine differences in growth trajectories following treatment. Results Comparison of postnatal growth curves from the time of treatment to 50 weeks PMA showed no significant differences in growth trajectories between groups after adjusting for the corresponding growth parameters at birth. Comparison of Bayley scores in patients with available data (n = 120) showed no significant differences. There was an increased risk of cerebral palsy in the IVB group after logistic regression adjusting for baseline confounders, but this did not retain statistical significance after applying the false discovery rate correction for multiple testing. Conclusion To our knowledge, this is the first large retrospective study to examine longitudinal growth in infants treated with IVB compared to controls. There were no significant differences in postnatal growth or neurodevelopmental outcomes between groups, which overall continue to support the safety of bevacizumab treatment for ROP.

No abstract

Background/aimsRetinal microvascular ischaemia may produce localised middle retinal disruption with corresponding scotoma, a phenomenon termed paracentral acute middle maculopathy (PAMM). Small chronic middle retinal atrophic lesions termed retinal ischaemic perivascular lesions (RIPLs) appear qualitatively similar to PAMM lesions and have recently been hypothesised to result specifically from PAMM. However, no studies have quantitatively demonstrated an ischaemic origin of RIPLs. We quantitatively investigated the pathophysiology of RIPLs and their relationship with PAMM with swept-source optical coherence tomography angiography (SS-OCTA).MethodsA total of 14 controls and 25 patients being evaluated for carotid artery stenosis (CAS) were enrolled. SS-OCTA imaging of each eye was taken. Projection-resolved en face 6 mm × 6 mm superficial capillary plexus (SCP) and deep capillary plexus (DCP) images were quantitatively analysed with two algorithms for changes in vessel linear density (VLD) and vessel tortuosity (VT) at RIPLs relative to both the immediately surrounding macula and the entire macula, as well as between eyes with RIPLs and eyes without RIPLs.ResultsAll controls and 22 of 25 CAS patients were included in the analysis. RIPLs demonstrated a localised decrease in DCP VLD in CAS patients and controls. RIPLs tended to show a localised decrease in SCP VLD in CAS patients but a localised increase in controls. No changes in VT were found. Eyes with RIPLs had VLD and VT similar to their RIPL-free fellow eyes.ConclusionRIPLs are associated with quantifiable local, but not global, ischaemia, supporting the idea of shared pathophysiology with classic PAMM lesions along a continuum of ischaemia severity.

No abstract