Iris rubeosis, severe respiratory failure and retinopathy of prematurity--case report

Case study reports for the first time about development of massive iris neovascular complication in course of retinopathy of prematurity related to systemic and ocular ischemic syndrome due to tracheostomy-requiring extremely severe premature respiratory failure.

Premature female, 950 grams birth weight, born from 17-year-old gravida 1, at 28 weeks' gestation by cesarean section due to premature placental abruption with threatening hemorrhages, with 1 to 5 Apgar score. The baby developed severe respiratory failure which required tracheostomy advanced bronchopulmonary dysplasia treated with steroids (BPD) and respiratory distress syndrome (RDS) with failure to extubate together with secondary ocular ischemia. All the mentioned with multifactorial organs complications (NEC, leucopenia, anemia, pneumonia, periventricular leucomalacia, electrolyte abnormalities and metabolic acidosis) resulted in massive peripupillary iris neovascularization (NVI) in both eyes coexisting with retinopathy of prematurity (ROP) in 38 weeks' PMA infant. Ultrasonography-B, slit-lamp and indirect fundus examinations with photography were used to document focusing ocular diagnosis. The previous retinopathy of prematurity screening examinations performed at standard intervals of time starting from four weeks of life, that is 32 weeks' PMA continuing every two weeks did not present typical lesions seen in retinopathy however in the second zone of retina slightly marked "plus sign" was visible. Ophthalmological examination at 38 weeks' PMA disclosed massive, round capillary dilatation of the minor arterial circle, spreading to the iris periphery form pupillary margin, and narrow pupils which did not fully react to mydriasis. The intraocular pressure was normal. Ultrasound examination revealed major thickening and echo increase of peripheral retina with its partial detachment.

Diode-laser pan-retinal photocoagulation and cryoapplication was performed leading to successful regression of neovascular anterior segment (rubeosis was no longer visible by slit-lamp examination) as well as inhibition of retinopathy of prematurity progression.

In described preterm infant, vasoactive molecules released by the ischemic retina may have induced vasodilation of iridal vessels, therefore increasing the perfusion of segments of the minor arterial circle, rendering them clinically visible by examination. It has been observed that hypoxia besides other inflammatory risk factors may have significant influence not only on the development of severe anterior-neovascular complications but also on rapid progression of advanced ROP stages with omission of typical clinical stages of ROP. Simultaneously retinal laser-panphotocoagulation with cryotheraphy seems to be the beneficial method for treatment of these complications. The therapy is effective and destroys the cells that produce Vascular Endothelial Growth Factor (VEGF) which is known to be the most important key factor in the progression of ROP.