Abstract

We report a case of a 5-year-old girl with persistent fetal vasculature who presented with unilateral acute angle closure caused by aqueous misdirection syndrome that was apparently associated with regression of cycloplegia. Initial treatment with topical steroids, anti-glaucomatous drops, and atropine showed insufficient control of the intraocular pressure (IOP). Surgical treatment with lensectomy and vitrectomy resulted in satisfactory IOP control and no complications at the 6-month follow-up.

Highlights

  • Persistent fetal vasculature (PFV) is a congenital anomaly of the eye caused by failure of regression of the primary vitreous during the embryonic period

  • This rare spectrum of disease, referred to as persistent hyperplastic primary vitreous, has a wide clinical spectrum, including elongated ciliary processes, shallow anterior chamber, retrolental fibrovascular membrane, cataract, vitreous membrane or stalk originating from the optic nerve, retinal fold, traction or de­tachment, and vitreous hemorrhage

  • Acute angle closure is a rare condition in children

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Summary

Introduction

Persistent fetal vasculature (PFV) is a congenital anomaly of the eye caused by failure of regression of the primary vitreous during the embryonic period. This rare spectrum of disease, referred to as persistent hyperplastic primary vitreous, has a wide clinical spectrum, including elongated ciliary processes, shallow anterior chamber, retrolental fibrovascular membrane, cataract, vitreous membrane or stalk originating from the optic nerve, retinal fold, traction or de­tachment, and vitreous hemorrhage. The mechanism of angle closure in PFV is not completely known, but includes an enlarged cataractous lens and contraction of the retrolental fibrovascular membrane with secondary anterior lens-iris diaphragm displacement[1].

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