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Copyright: © 2016 Trivizki O, et al.
Journal of Ophthalmic Diseases
Case Report
Open Access
Persistent Fetal Vasculature with Atypical Presentation
Trivizki O1*, Mezad-Koursh D1 and Stolovich C2
1
Department of Ophthalmology, Tel Aviv Sourasky Medical Center, Tel-Aviv, Israel
2
Sackler Faculty of Medicine, TelAviv University, Tel Aviv, Israel
Received Date: May 20, 2016, Accepted Date: August 31, 2016, Published Date: September 15, 2016.
*Corresponding author: Omer Trivizki, Department of Ophthalmology, Tel Aviv Sourasky Medical Center, Tel Aviv 6423906, Israel. Tel: 9723-6973-408;
Fax: 9723-6973-408; E-mail: [email protected]
Abstract
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; MicroOphthalmia; Cataract
Abbreviations
PFV: Persistent Fetal Vasculature.
Introduction
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.
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.
Figure 1: Anterior segment slit-lamp photograph showing leukocoria.
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 slitlamp 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).
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).
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
Discussion
PFV is a disorder of failure in ocular development due to
incomplete regression of the fetal hyaloid vasculature [1,2]. It is
J Ophth Dis
Figure 2: An ultrasound biomicroscope study showing an echogenic
band extending from the posterior surface of the lens capsule to the
optic disc.
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,
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J Ophth Dis
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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*Corresponding author: Omer Trivizki, Department of Ophthalmology, Tel Aviv Sourasky Medical Center, Tel Aviv 6423906, Israel. Tel: 9723-6973408; Fax: 9723-6973-408; E-mail: [email protected]
Received Date: May 20, 2016, Accepted Date: August 31, 2016, Published Date: September 15, 2016.
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.
Citation: Trivizki O, Mezad-Koursh D, Stolovich C (2016) Persistent Fetal Vasculature with Atypical Presentation. J Ophth Dis 1(1): 105.
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Citation: Trivizki O, Mezad-Koursh D, Stolovich C (2016) Persistent Fetal Vasculature with Atypical Presentation. J Ophth
Dis 1(1): 105.
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