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Persistent vasa hyaloidea propria/retinae in familial exudative vitreoretinopathy
Eric D Gaier1, Yoshihiro Yonekawa2
1Department of Ophthalmology, Boston Children's Hospital, Boston, Massachusetts; Department of Ophthalmology, Massachusetts Eye & Ear, Boston, Massachusetts; Department of Ophthalmology, Harvard Medical School, Boston, Massachusetts; Picower Institute for Learning and Memory, Department of Brain and Cognitive Sciences, Massachusetts Institute of Technology, Cambridge, Massachusetts.
Insights
Persistent vasa hyaloidea propria/retinae can cause retinal detachment in infants. Surgical transection of these fetal vessels successfully treated a case of stage 4 familial exudative vitreoretinopathy.
Area of Science:
- Ophthalmology
- Vascular Biology
- Pediatric Medicine
Background:
- The vasa hyaloidea propria are fetal vasculature remnants within the vitreous.
- Persistence of these vessels can lead to various ocular pathologies.
- Familial exudative vitreoretinopathy (FEVR) is a genetic disorder affecting retinal vascular development.
Purpose of the Study:
- To describe a case of stage 4 FEVR caused by persistent vasa hyaloidea propria/retinae.
- To illustrate the diagnostic findings and surgical management of this condition.
Main Methods:
- Case presentation of a 4-month-old girl with FEVR.
- Diagnostic confirmation using fluorescein angiography.
- Surgical intervention involving vitreoretinal surgery.
Main Results:
- The patient presented with stage 4 FEVR and ectopic retinal vessels.
- Fluorescein angiography confirmed persistent vasa hyaloidea propria/retinae with retinal contact.
- Vitreoretinal surgery successfully addressed the retinal detachment by transecting the persistent vessel stalks.
Conclusions:
- Persistent vasa hyaloidea propria/retinae is a significant cause of retinal detachment in FEVR.
- Surgical management can be effective in treating FEVR associated with these persistent fetal vessels.
Abstract:
The vasa hyaloidea propria, a component of the fetal hyaloidal vasculature, is characterized by multiple persistent fetal vasculatures branching into the vitreous. We present a 4-month-old girl with stage 4 familial exudative vitreoretinopathy, with multiple ectopic retinal vessels extending into the vitreous, confirmed with fluorescein angiography, which was consistent with persistent vasa hyaloidea propia/retinae making contact with the retina. The patient underwent vitreoretinal surgery to address the retinal detachment, during which the patent stalks of the persistent vasa hyaloidea propia/retinae were transected.
