ABSTRACT
PURPOSE: To investigate persistent avascular retina in infants with type 2 retinopathy of permaturity (ROP) that persisted after 45 weeks' post-menstrual age when regular ROP screening ceased. METHODS: A prospective observational study where fundus fluorescein angiography (FFA) was completed on consecutive infants who had a history of type 2 ROP and avascular retina during ROP screening that persisted after 45 weeks' post-menstrual age. RESULTS: FFA was completed on 72 eyes of 36 infants (53% male), with a mean gestational age of 26.0 ± 2.2 weeks and a mean birth weight of 834.6 ± 216.3 grams. The mean age at discharge from ROP screening was 47.6 weeks' post-menstrual age. All patients had type 2 ROP at the worst stage of their disease, with predominantly stage 2 disease. FFA was performed at a mean age of 18.8 ± 10.3 months post-menstrual age. All patients had detectable avascular retina in peripheral zone II or III on FFA. Peripheral vessel leakage was present in 3 eyes of 2 infants (5.5%), who both subsequently received peripheral laser treatment. CONCLUSIONS: Premature infants with type 2 ROP may have persistent peripheral avascular retina with unknown long-term ocular complications, which can present management dilemmas. Retinal FFA is recommended to determine retinal ischemia and aid decision making for treatment in these cases. [J Pediatr Ophthalmol Strabismus. 2019;56(4):222-228.].
Subject(s)
Decision Making , Laser Coagulation , Retina/abnormalities , Retinal Vessels/abnormalities , Retinopathy of Prematurity/diagnosis , Visual Acuity , Female , Fluorescein Angiography , Fundus Oculi , Gestational Age , Humans , Infant, Newborn , Male , Prospective Studies , Retina/diagnostic imaging , Retinal Vessels/diagnostic imaging , Retinopathy of Prematurity/therapy , Slit Lamp MicroscopyABSTRACT
BACKGROUND: Leigh syndrome, French Canadian type is a rare neurodegenerative disease. To our knowledge, there have been no studies based on ocular findings published for this disease. The purpose of this study is to describe ophthalmic findings in these patients. PATIENTS: Six patients genetically identified as having the syndrome were included in this study. METHODS: Four patients had an ophthalmic examination with an ophthalmologist including evaluation of visual acuity, extraocular motility and lid position, orthoptic workup, evaluation of stereopsis, refraction, evaluation of pupils, color vision, slit-lamp biomicroscopy, measurement of intraocular pressure, and fundoscopy. Two patients had a chart review. RESULTS: Visual acuity ranged from 0.00 logmar to 1.55 logmar. Extraocular motility abnormalities and ptosis were noted in half of the patients. Strabismus was present in the entire cohort, and stereopsis was absent in half of these patients. Amblyopia was noted in 83% of individuals and suppression in 33%. Only one patient had nystagmus. Refraction varied throughout patients. It included severe hyperopia, myopia, astigmatism, and significant anisometropia. Pupils, anterior segment, fundus, and color vision were normal in all patients. Intraocular pressure was slightly elevated in one patient. CONCLUSION: Patients with Leigh syndrome, French Canadian type display a variety of ophthalmic findings, and screening at a young age is recommended.
