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Paeds Casesfetal-neonatal-and-perinatal

Paeds Cases · fetal-neonatal-and-perinatal

Retinopathy of prematurity

Clinical case of an extremely preterm infant reaching the Type 1 ROP treatment threshold.

long-case-clinical-reasoning2 min readVerification in progress

Target exams

RACP DCEMRCPCH ClinicalABP General Pediatrics
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Study tools

Target exams

RACP DCEMRCPCH ClinicalABP General Pediatrics
Prompt
A 25-week gestation infant (birth weight 640 g), now 33 weeks postmenstrual age, has Zone I stage 3 ROP with prominent plus disease on routine screening.

Clinical Case

History

This infant was born at 25 weeks and 640 g to a 29-year-old primigravida after preterm labour complicated by histological chorioamnionitis. He was intubated in the delivery room and received two doses of surfactant for respiratory distress syndrome. His NICU course was marked by fluctuating oxygen saturations, late-onset staphylococcal sepsis at day 12, two packed red cell transfusions for anaemia of prematurity, and growth that remained below the 10th centile despite fortification [4].

Examination at 33 weeks postmenstrual age

Routine retinal screening showed, in both eyes, Zone I stage 3 disease with prominent plus disease and flat neovascularisation at an ill-defined vascular-avascular junction, extending across five clock-hours temporally [1].

Key Questions

1. What is this infant's classification, and why does it matter? This is Zone I stage 3 ROP with plus disease, which meets Type 1 criteria and is the treatment threshold defined by the ETROP trial [2]. The prominent plus disease and ill-defined junction with flat neovascularisation in Zone I also raise concern for aggressive ROP, which can progress to detachment within days, so this is a sight-threatening emergency [1].

2. How should this infant be treated? Type 1 ROP must be treated within 72 hours [2]. Because the disease is in Zone I, intravitreal anti-VEGF therapy is often preferred over laser, as the BEAT-ROP trial showed bevacizumab was superior to laser for Zone I stage 3 disease with plus [3]. Laser remains an alternative where anti-VEGF is unsuitable or follow-up cannot be guaranteed [2].

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References4ShowHide
  1. [1]Chiang MF, Quinn GE, Fielder AR, et al International Classification of Retinopathy of Prematurity, Third Edition Ophthalmology, 2021.PMID 34247850
  2. [2]Early Treatment for Retinopathy of Prematurity Cooperative Group Revised indications for the treatment of retinopathy of prematurity: results of the early treatment for retinopathy of prematurity randomized trial Arch Ophthalmol, 2003.PMID 14662586
  3. [3]Mintz-Hittner HA, Kennedy KA, Chuang AZ; BEAT-ROP Cooperative Group Efficacy of intravitreal bevacizumab for stage 3+ retinopathy of prematurity N Engl J Med, 2011.PMID 21323540
  4. [4]Fierson WM Screening Examination of Premature Infants for Retinopathy of Prematurity Pediatrics, 2018.PMID 30478242
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