Journal of Ophthalmic Diseases

Persistent Fetal Vasculature with Atypical Presentation

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Published Date: September 15, 2016

Persistent Fetal Vasculature with Atypical Presentation

Trivizki O1*, Mezad-Koursh D1, and Stolovich C2

1Department of Ophthalmology, Tel Aviv Sourasky Medical Center, Tel-Aviv, Israel

2Sackler Faculty of Medicine, TelAviv University, Tel Aviv, Israel

*Corresponding Authors: Omer Trivizki, Department of Ophthalmology, Tel Aviv Sourasky Medical Center, Tel Aviv 6423906, Israel. Tel: 9723-6973-408; Fax: 9723-6973-408; E-mail:


Citation: Trivizki O, Mezad-Koursh D, Stolovich C (2016) Persistent Fetal Vasculature with Atypical Presentation. J Ophth Dis 1(1): 105.




Persistent fetal vasculature is an uncommon condition, presenting clinically as Leukocoria (white pupillary reflex), micro-ophthalmia, and cataract. The authors report a case of an infant with atypical normal corneal diameter and long axial length sizes.

Keywords: Persistent Fetal Vasculature; Leukocoria; Micro-Ophthalmia; Cataract


PFV: Persistent Fetal Vasculature.

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Persistent fetal vasculature (PFV) is an uncommon condition of failure in ocular development due to incomplete regression of the fetal hyaloid vasculature. Here we report of a unique presentation of PFV.

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Case Report


A 21-month-old boy presented to the Tel Aviv Sourasky Medical Center Pediatric Ophthalmic Unit due to suspected high myopia, unilateral right leukocoria and a new right eye strabismus. He was born at term following an uneventful pregnancy and birth and was otherwise healthy. Family history was unremarkable.

At initial visit he presented with signs of amblyopia in his right eye by fixating pattern, a moderate right esotropia (20PD) was diagnosed in the primary position. Pupil were equal and reactive to light, there was no palpable mass or swelling. He was systemically well with no other focal neurological signs on slit-lamp biomicropscopy examination a central posterior sub capsular cataract was seen (Figure 1). Due to the media opacity a fundus examination to the right eye was unsuitable. The left eye including the anterior and posterior part appeared to be normal except hypermetropia of 3.50 diopter (D).


Figure 1: Anterior segment slit-lamp photograph showing leukocoria.


As mentioned, since there was no clear view of the fundus, a B-scan ultrasound study was performed in which a combined PFV was found (Figure 2). Under anesthesia, the horizontal cornea diameter was 12 mm bilaterally (normal 10.00-12.00 mm), and the axial length of the left eye was 20.76 mm on the left eye and 23.55 mm on the right eye (average of 10 serial measurements with 0.10 mm standard deviation, OcuScan RxP, Alcon; normal values to this age, 19.70 - 23.00 mm1).


Figure 2: An ultrasound biomicroscope study showing an echogenic band extending from the posterior surface of the lens capsule to the optic disc.


Lensectomy, posterior capsulotomy, core vitrectomy, removal of the remnant fibrovascular stalk and intraocular lens implantation of Acrys of 14.5 diopter lens were successfully performed

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PFV is a disorder of failure in ocular development due to incomplete regression of the fetal hyaloid vasculature [1,2]. It is usually divided into anterior, posterior or combined manifestation, and is characterized by the presence of a vascular stalk between the lens capsule and the optic disc [3]. The condition is usually unilateral and bilaterally is rare (< 3%) [4]. Anterior PFV is considered the most frequent cause of unilateral lens opacification, and it is associated with both short axial length and reduced cornea size. In posterior PHPV is also associated with reduced cornea size and more than two thirds of cases are microphthalmic [5,6]. A grey-scale ultrasonographic study in an eye with PFV will show an echogenic band extending from the posterior surface of the lens capsule to the optic disc [1].

Descriptions of normal axial length and normal corneal diameter in PFV patients are sparse [7–9]. Most affected individuals have a mild form of the disease, and some of them do not have a classic leukocoria presentation and therefore tend to seek help later on in life [7–9]. The herein described classic young age presentation of PFV is unique for having an atypically longer than usual and then the second normal eye axial length and an atypically normal corneal diameter. To our knowledge this is the first report describing a unilateral case of classic presentation of PFV with a longer axial length in the affected eye. 

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  1. Capozzi P, Morini C, Piga S, Cuttini M, Vadalà P. Corneal curvature and axial length values in children with congenital/infantile cataract in the first 42 months of life. Invest Ophthalmol Vis Sci. 2008;49(11):4774–8. doi:10.1167/iovs.07-1564.
  2. Goldberg MF. Persistent fetal vasculature (PFV): an integrated interpretation of signs and symptoms associated with persistent hyperplastic primary vitreous (PHPV). LIV Edward Jackson Memorial Lecture. Am J Ophthalmol. 1997;124(5):587–626.
  3. Cerón O, Lou PL, Kroll AJ, Walton DS. The vitreo-retinal manifestations of persistent hyperplasic primary vitreous (PHPV) and their management. Int Ophthalmol Clin. 2008;48(2):53–62. doi:10.1097/IIO.0b013e3181695980.
  4. Pollard ZF. Persistent hyperplastic primary vitreous: diagnosis, treatment and results. Trans Am Ophthalmol Soc. 1997;95:487–549.
  5. Bardakjian T, Weiss A, Schneider AS. Anophthalmia/Microphthalmia Overview. 2006.
  6. Haddad R, Font RL, Reeser F. Persistent hyperplastic primary vitreous. A clinicopathologic study of 62 cases and review of the literature. Surv Ophthalmol. 23(2):123–34.
  7. Darusman KR, Aquino MCD, Thamboo TP, Wong IB. A case of persistent fetal vasculature with atypical presentation. Eye (Lond). 2011;25(11):1521–2. doi:10.1038/eye.2011.206.
  8. Küçükevcilio?lu M, Melih OM,Mutlu FM, Altinsoy HI, Erdurman FC, Hürmeriç V. Persistent Fetal Vasculature with A Long Axial Length: Possible Inhibitory Effect on Globe Elongation. Journal of Glaucoma Cataract. 2011; 6(4):254-256.
  9. Steele G, Peters R. Persistent hyperplastic primary vitreous with myopia: a case study. J Am Optom Assoc. 1999;70(9):593–7

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Copyright: © 2016 Trivizki O, et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.