Case Archive
Pediatric Ophthalmology
Ref. # 357501

An infant was born at an estimated gestational age (EGA) of 26 weeks, weighing 1000 grams with Apgars of 5, 5 and 9. His hospitalization was complicated by Respiratory Distress Syndrome (RDS), prematurity anemia, aspiration pneumonia, and bilateral periventricular leukomalacia. He had oxygen by nasal cannula or ventilator for the first 35 days and after discharge. The infant's vision was impacted, leading to evaluation by a pediatric ophthalmologist first at the hospital and then at his office. Initial impression was immature retina peripherally and no Retinopathy of Prematurity (ROP). After two more bi-weekly exams, the findings were Stage 2 ROP, drawings showing a few "popcorn" lesions nasally, and high myopia. A return visit was scheduled for two weeks. The appointment was missed. The parents were not informed the child's condition mandated immediate evaluation. At the appointment the next week, the findings were Stage 2 ROP with plus disease "resolving nicely," vascularization passed the demarcation line to periphery, and high myopia. The child was then examined by a retinal specialist four days later. The diagnosis was ROP stage 3 or 4, Zone 2, as well as tractional retinal detachments (TRD) in both eyes. Eleven days later a laser retinoplexy showed advanced fibrovascular proliferation with vitrious hemorrhage in both eyes. Susbsequent surgeries were performed. The child is blind in both eyes with a poor prognosis. A medQuest pediatric ophthalmologist reported ROP is a multifactorial disease of premature children, classified by location (Zone, 1, 2 and 3) and severity (Stage 1-4). 85% of ROP is transient disease with spontaneous regression. Approximately 6% of children reach threshold, 20% are prethreshold, and 60% of children have some ROP post partum. Extra risk factors include a birth weight under 750 grams, estimated gestational age (EGA) of less than 28 weeks, oxygen use for more than three weeks, and Caucasian race. Threshold ROP is considered a certain amount of Stage 3 ROP with plus disease (certain tortuous characteristics of the vessels). Up to 50% of children with severe ROP have high myopia. Although ROP is an unpredictable and often rapidly progressive disease, it is very unlikely it transformed in this infant's case from Stage 2 to Stage 4 in the four-day interval between the pediatric ophthalmologist's last exam and the retinal specialist's first exam. The ROP likely reached threshold stage several weeks earlier, causing the TRD. The failure to diagnose and treat threshold ROP in a timely fashion greatly decreased the child's chances of developing useful vision.

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