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Central retinal artery and vein occlusion as a complication of persistent hyaloid artery - a case report
Mirjana Bjeloš1,2,3, Ana Križanović1,3, Mladen Bušić4,5,6
1Department of Ophthalmology, Reference Centre for Paediatric Ophthalmology and Strabismus of the Ministry of Health of the Republic of Croatia, Sveti Duh University Hospital, Sveti Duh 64, Zagreb, Croatia.
Insights
Persistent hyaloid artery (PHA) can lead to central retinal artery occlusion (CRAO) and central retinal vein occlusion (CRVO). Early diagnosis via fluorescein angiography and prompt surgical intervention are crucial for managing this rare complication.
Area of Science:
- Ophthalmology
- Vascular Medicine
- Embryology
Background:
- Persistent hyaloid artery (PHA) is a rare congenital anomaly resulting from failed apoptosis during gestation.
- PHA can be detected during neonatal red reflex screening.
- This case report highlights a novel complication of PHA.
Observation:
- A six-year-old male presented with acute eye symptoms, diagnosed with neovascular glaucoma, CRVO, and CRAO via fluorescein angiography.
- Examination revealed a non-reactive pupil and a tubular remnant of the hyaloid artery in the vitreous.
- The patient had no significant family history or other remarkable medical findings.
Findings:
- The study hypothesizes that twisting of the PHA caused torsion, leading to CRAO first.
- Subsequent venous stasis from reduced arterial inflow likely resulted in CRVO.
- The development of liquid vitreous around age four may facilitate PHA rotation and subsequent vascular events.
Implications:
- Early detection of PHA is crucial for identifying at-risk individuals.
- Fluorescein angiography is recommended for PHAs connected to the retinal artery.
- Prompt surgical treatment should be considered for PHA to prevent severe visual complications.
Background:
In this case report, we present for the first time central retinal artery occlusion (CRAO) and central retinal vein occlusion (CRVO) as a complication of persistent hyaloid artery (PHA).
Case Presentation:
In August 2019, a six-year-old male patient manifested right eye (RE) excessive tearing, conjunctival injection and pain. On examination, RE demonstrated light perception and intraocular pressure of 36 mmHg. The diagnoses of neovascular glaucoma, CRVO and CRAO were established as affirmed with fluorescein angiography (FA). PHA was not reported. Extensive work-up and family history were unremarkable. The child was born on term after uncomplicated twin pregnancy. In December 2019, he was referred to our Centre. Transillumination revealed fully dilated, non-reactive RE pupil, clear lens and tubular remnant of HA containing blood cells in its lumen freely rotating in the anterior vitreous.
Conclusions:
PHA results from failure of apoptosis during gestation. It can easily be observed during the red reflex screening at neonatal wards. We hypothesized that PHA twisting led to torsion of the residual primordial common bulb, branching off to HA and CRA with CRAO occurring first. The consequential CRVO presumably advanced by venous stasis due to decrease in arterial inflow. Liquid vitreous appears as early as 4 years of age enabling PHA to whirl more freely. Thus, in case of PHA, we advocate FA to be performed and if connection with retinal artery is proven, parents should be informed on the possible devastating complications and prompt surgical treatment should be considered.
