Atypical anterior persistent hyperplastic primary vitreous: report of a rare case

Abstract

Background: Persistent hyperplastic primary vitreous (PHPV) is a congenital form of vitreous dysplasia that can be categorized into anterior, posterior, and mixed types according to the affected location within the eye. Definitive diagnoses of PHPV are usually made based on B-mode ultrasound, optical coherence tomography (OCT), and Doppler ultrasound findings. In this report, we discuss the case of a 7-year-old boy in whom a definitive diagnosis of atypical anterior PHPV was possible based on intraoperative observations, pathological findings, and the results of ophthalmic examination. Case presentation: A 7-year-old boy presented with leukocoria and acute glaucoma in his right eye. Imaging suggested characteristics of mixed PHPV. Surgical treatment and pathological examination were performed due to the presence of acute glaucoma and abnormal lens morphology. Typical signs of posterior PHPV (e.g., eyeball shrinkage, the presence of vascular membranes connected to the optic disc, etc.) were not observed. However, there were abundant fibrous vascular membranes around the lens. Pathological examination revealed fibrocyte proliferation in the lens and capsular tissue. Intraoperative findings were used in conjunction with the results of pathological and ophthalmological examinations to make the final diagnosis of anterior PHPV. Conclusion: The course and characteristics of PHPV can be unpredictable, and it is often the case that a clear diagnosis cannot be obtained based on clinical characteristics and typical imaging examinations alone. Further surgical treatment and pathological examination may aid in establishing a final diagnosis. In addition to treating the complications of PHPV (e.g., glaucoma), surgery may improve eye appearance and restore visual function to some degree.

Surgical Treatment of Persistent Hyperplastic Primary Vitreous: A Case Report

“…The remnant vessel connects the optic nerve's head posteriorly to anterior ocular structures, such as the lens and ciliary process, and its manifestation varies among patients. Based on the affected ocular structures, it can be divided into three types: anterior, posterior, and combined (or mixed) types [3] with a distribution of 25, 12, and 63%, respectively, among all affected subjects [4]. Anterior PHPV is usually associated with an opacity of the lens or posterior capsule cortex, continuous fibrovascular membrane hyperplasia behind the lens, as well as elongation of the ciliary body.…”

The Outcome of Manual Small Incision Cataract Surgery and Anterior Vitrectomy for Persistent Fetal Vasculature in an 18-Year-Old Woman: A One-Year Follow-Up

“…The posterior pole was visualized postoperatively and the optic disc and macula showed normal findings. When the visual acuity after surgical intervention for PFV is better than 1/60, it can be referred to as “useful vision”; when it is 1/60 or less, it is considered to be “poor vision” [ 7 ]. For the index case, the unaided and aided visual acuity over the follow-up period remained 6/60 and 6/36, respectively, despite amblyopia therapy by patching of the better eye.…”

“…Morphologically, PFV can be anterior, posterior, or a combination of both [ 6 ]. Anterior PFV is characterized by one, some, or all of the following features: a retrolental opacity (Mittendorf dot), elongated ciliary processes, cataract, shallow anterior chamber, poor pupil dilation, microphthalmos, microcornea, and glaucoma [ 7 ] while the posterior PFV has the following features: the stalk of tissue attached to the optic nerve (Bergmeister's papillae), retinal folds, dysplasia, retinal detachment, and varying degrees of optic nerve dysplasia [ 8 , 9 ]. The prevalence rate of PFV is unknown, however, published articles suggest that it is responsible for about 5% of all cases of blindness [ 10 ].…”