REVIEW ARTICLE
Persistent Fetal Vasculature
Chonglin Chen, MD, Hu Xiao, MD, and Xiaoyan Ding, MD, PhD
Abstract: During development, the fetal vasculature nourishes
the developing lens and retina, and it subsequently regresses after the
formation of the retinal vessels. Persistent fetal vasculature (PFV)
occurs as a result of a failure of fetal ocular vasculature to undergo
normal programmed involution, which leads to blindness or serious
loss of vision. Persistent fetal vasculature is responsible for as much
as 5% of childhood blindness in western countries. The regulatory
mechanisms responsible for fetal vascular regress remain obscure, as
do the underlying causes of the failure of regression. Because of recent
advancements in microinvasive surgical techniques, the early treatment
of PFV has become safer and more effective, thus paving the way for the
development of a future new treatment strategy. In this review, clinical
and imaging manifestations of PFV and the progress in the treatment of
PFV are highlighted.
Key Words: child, persistent fetal vasculature, vitreous body
(Asia Pac J Ophthalmol (Phila) 2019;8:86–95)
P ersistent fetal vasculature (PFV) is a congenital ocular
anomaly in which the embryonic hyaloid vasculature network
fails to normally regress partially or completely.1,2 It was first
reported by Cloquet3 in 1818, since then it has been called by
various names, including persistent posterior fetal fibrovascular
sheath of the lens, persistent tunica vasculosa lentis (TVL),
persistent hyperplastic vitreous (PHPV), congenital retinal
septum, and ablation falciform.1,4 In 1997, Goldberg4 proposed
to place all the anterior, posterior, and combined manifestations
into the same category with a general name—PFV. This term
emphasizes the etiology of the disorder, incorporates the full
extent of the disease, and offers a better description of the
various clinical manifestations associated with the failure of fetal
vasculature regression. The defects include glaucoma, cataract,
falciform retinal folds, funnel- or stalk-shaped retinal detachment,
spontaneous fundus hemorrhage, and a congenitally small eye.
From the State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center,
Sun Yat-Sen University, Guangzhou, China.
Submitted February 6, 2018; accepted August 1, 2018.
Supported by grants from the Fundamental Research Funds of State Key Laboratory
of Ophthalmology, research funds of Sun Yat-sen University (15ykjc22d;
Guangzhou, Guangdong, China), and Science and Technology Program
Guangzhou, China (201803010031; Guangzhou, Guangdong, China).
Reprints: Xiaoyan Ding, State Key Laboratory of Ophthalmology, Zhongshan
Ophthalmic Center, Sun Yat-Sen University, Guangzhou, 510060, China.
E-mail: Dingxiaoyan@gzzoc.com.
Copyright © 2019 by Asia Pacific Academy of Ophthalmology
ISSN: 2162-0989
DOI: 10.22608/APO.201854
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As the term PFV reflects a more accurate description of anatomic
and pathologic features, it has gradually replaced the term PHPV.
EMBRYOLOGY
During early embryogenesis, the intraocular fetal vascular
system is critical for the development of the lens, vitreous, and
retina. The vascular system arises from the optic nerve head,
extends through the central vitreous, surrounds the developing
crystalline lens, and finally reaches and nourishes the anterior
segment of the eye. This system comprises 3 distinct anatomic
structures: the vasa Hyaloidea propria (VHP), which consists
of branches of the hyaloid vessels in the vitreous closest to the
retina; the TVL, which consists of branches of the hyaloid artery
covering the posterior hemisphere of the lens; and the pupillary
membrane, which consists of branches that cover the more
anterior part of the lens. Timely regression of the fetal vascular
system is very important for the development of a clear optical
path. The VHP is the first element of the hyaloid vasculature
that undergoes regression, followed by the TVL, the pupillary
membrane, and, finally, the hyaloid stalk. When retinal vessels
begin to appear, the fetal vasculature normally regresses, shrinks,
and finally disappears before birth in humans or within the first
few weeks after birth in rodents. Normally, only an acellular
hyaloid canal, called the Cloquet canal, is left; it is shaped like
a funnel, with a narrow end anterior to the optic nerve head and
a wide end posterior to the lens. Any abnormalities along the
involution pathway can produce different clinical manifestations
of PFV, which results from the partial or complete failure of
vascular regression.
PATHOLOGY
The reason for the persistence of the vasculature is still
unknown. In a histologic review, glial cells were contained
abundantly in retrolental fibrovascular stalks.5 Thus, the glial
and vascular components of the stalk cause anteroposterior
traction on the peripapillary retina, leading to macular dragging
and tractional retinal detachment. In some cases, fibrovascular
dysplasia is progressive, leading to corneal clouding, secondary
angle-closure glaucoma, cataract, intraocular hemorrhage, retinal
detachment, and even phthisis bulbi.1,4 Most PFVs in humans
are sporadic.6–8 Up to now, few reports have been published on
inherited forms of PFV. A few loci for isolated PFV have been
mapped, but no candidate gene has been reported in humans. In
2001, Khaliq et al7 reported the linkage of autosomal recessive
PFV locus to 10q11-q21 by microsatellite mapping in a large
inbred Pakistani pedigree. Unlike the case in humans, however,
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a number of gene mutations have been shown to cause bilateral
PFV in animal models.9,10 The present literature shows at least
15 different genes in knock-out (KO)/transgenic mouse models
that exhibit the clinical features of PFV, including CrybetaA3/
A1, Lrp5, and Fzd5 KO mouse. However, most of these genes
were notably reported to be associated with familial exudative
vitreoretinopathy (FEVR). This discrepancy might be explained
by the overlap of clinical phenotypes between FEVR and PFV.
CLASSIFICATION
Although the exact prevalence of PFV is still unknown, it
should not be considered a very rare disease. It accounts for about
5% of blindness in childhood in the United States.7 Notably,
95% of PFV cases are affected unilaterally. Although rare,
bilateral cases have been reported and are usually associated with
congenital syndromes, such as trisomy13,11 trisomy 15,12 Aicardi
syndrome,13 Norrie disease,14 Walker–Warburg syndrome,15 and
osteoporosis–pseudoglioma (OPPG) syndrome.16
Traditionally, PFV could be divided into 3 categories based
on the location of the vascular abnormalities—purely anterior,
purely posterior, and combined PFV.17 Purely anterior PFV is
relatively common, accounting for approximately 25% of cases
and is characterized by cataracts, retrolental opacity, and, in some
pediatric cases, a shallow anterior chamber and elongation of
ciliary processes. Secondary angle-closure glaucoma, resulting
from a swollen lens, may occur in a few cases. Purely posterior
PFV mainly involves the vitreous and the retina and accounts
for 12% of PFV patients. It may manifest as a stalk from the
optic nerve, retinal proliferative membrane, retinal fold, retinal
detachment, or optic nerve hypoplasia. Combined PFV, involving
both the anterior and posterior segments and is the most common
type, accounts for about 60% of all cases.6 Of these, 37% to
46% eventually progress to no-light-perception vision if the
condition is left untreated.18–20 Combined PFV may develop into
corneal opacification, secondary angle-closure glaucoma, corneal
clouding, or spontaneous intraocular bleeding.
CLINICAL MANIFESTATIONS
The presentation of PFV is highly variable. Each of the
anatomic abnormalities is associated with partial or complete
persistence of the fetal vasculature and thus can be considered
a limited expression of the complete PFV syndrome.4–6,9 We
describe herein the most common clinical variants, from anterior
to posterior, according to the classification and categorization of
Goldberg.4
Anterior Persistent Fetal Vasculature
Iridohyaloid Blood Vessels
Iridohyaloid blood vessels are caused by the regression
failure of the anterior TVL.21 These vessels lead to the appearance
of radial vessels lying superficially on the iris and/or hairpin
loops on the collarette. In some cases, limbal connective tissue
malformation can also be detected in the same meridian. Visual
acuity is usually unaffected.
Persistent Pupillary Membranes
A more clinically descriptive term, persistent pupillary loops
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Persistent Fetal Vasculature
and strands, was used in prior literature. These loops and strands
are remnants of the anterior TVL, which is the blood supply
during lens development of the fetus. Remnants of the capillaries
may persist as small strands attached to the collarette of the iris,
or the membrane-like lesion located in the pupillary area, thus
the term persistent pupillary membrane (Fig. 1). In some cases,
congenital cataract or retrolental fibrous tissue can be noted in the
same eye, which is very helpful in confirming the diagnosis of
PFV.22–25 In patients with partial persistent pupillary membrane,
visual acuity is usually unaffected. Occasionally, visual acuity is
compromised as the membrane becomes thick or intact, which
causes deprivation amblyopia. In these cases, mydriatic agents,
surgical excision, or laser should be considered to remove the
membrane from the visual axis.
Mittendorf Dot
The Mitterndorf dot is a white dot located on the posterior
lens capsule, usually about 0.5 mm nasal to the posterior pole. It
is caused by the incomplete regression of the hyaloid artery. As
the well-defined small dot rarely affects vision, no treatment is
needed. The Mitterndorf dot can be found in 0.7% to 2.0% of the
general population.26
Retrolental Membrane
The retrolental membrane, classically called PHPV, is
characterized by fibrous membranes located in the retrolental
space. It is caused by the failure of regression of the posterior TVL
(Fig. 2). Typically, the retrolental membrane is white or pink,
distinguishing it from the yellow exudation found in Coats disease
and FEVR, or the snow-white calcification in retinoblastoma. The
area of the retrolental membrane varies widely. It may be as small
as a dot in some cases, or it may occupy the entire posterior surface
of the lens in others. The lens itself varies from completely clear
to severe opacification. Elongated ciliary processes can be found,
and these are due to the proliferation and concentric traction of
the remnant posterior TVL (Fig. 3).
FIGURE 1. Persistent pupillary membrane. A 2-year-old boy with a
visible pupillary membrane and iris dysplasia in the left eye.
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Leukocoria
Leukocoria is one of the most commonly reported symptoms
by the parents of children with PFV. The “white” pupil is mainly
due to the retrolental membrane. In most cases, retrolental fibrous
membranes are attached to the posterior lens capsule, leading
to the appearance of cataract. Occasionally, the posterior lens
capsule ruptures and thus causes opacification of the lens, which
is secondary to the traction of the persistent hyaloid artery.
Interestingly, PFV is well known to be one of the most
common causes of unilateral congenital cataracts. A meta-
analysis showed that approximately 11% to 30% of pediatric
unilateral cataracts cases were associated with PFV.23 Haargaard
et al27 even reported that more than half (57%) of cataract cases,
which involve 0- to 17-year-olds, were due to PFV. Moreover,
in light of the broad definition of PFV, Müllner-Eidenböck et
al22 found signs of PFV in all cases with congenital unilateral
cataracts during surgical treatment. These cases usually manifest
FIGURE 2. An 11-month-old girl with a yellowish white fibrous
membrane covering the entire posterior surface of the lens in her
left eye. There are vessels extending on the membrane. The fundus is
invisible. However, the lens is clear.
FIGURE 3. A 1.5-year-old girl with a yellowish-white retrolenticular
fibrovascular membrane in her right eye, and the ciliary processes are
centrally dragged and elongated.
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Asia-Pacific Journal of Ophthalmology • Volume 8, Number 1, January/February 2019
as persistent vasculature immediately posterior to the lens,
posterior capsular plaque with ghost vessels, proliferative fibrous
membrane, and even defects of the posterior capsule resulting
from traction. As the central part of the posterior capsule is very
close to the nodal point of the eye, even a small or mild opacity
on the nodal point may lead to deprivation amblyopia and may
significantly block visual development. Therefore, early surgical
intervention, followed by postoperative amblyopia therapy, is
necessary for visual recovery in children with anterior PFV.
Posterior Persistent Fetal Vasculature
Bergmeister Papilla
A Bergmeister papilla manifests as a membranous or
short band-like lesion attached to the optic disc, which is the
incomplete regression of the posterior part of the hyaloid artery.28
The Bergmeister papilla itself will not affect visual function, if
the remnant causes no macular traction.
Retinal Folds
In some cases, PFV is also accompanied by retinal folds.
Different from temporal predisposition, retinal folds associated
with PFV may occur in any quadrant, but they still have an
inferotemporal tendency.29 In most patients, a normal anterior
chamber and clear lens are present. However, in some advanced
cases, the vitreous proliferates along the Cloquet canal, thus
leading to retinal folds or even complete tractional retinal
detachment (Fig. 4).
Combined Persistent Fetal Vasculature
Combined PFV is the most common but complicated form
in this broad disease spectrum.30 The prognosis is quite poor;
sometimes global enucleation is required (Fig. 5).
Congenital Hyaloid Stalk and Tent-Shaped Retinal
Detachment
The primary vitreous containing the hyaloid artery is
located between the optic disc and lens posterior capsule, and
it proliferates and adheres to the retina, causing partial retinal
FIGURE 4. Retinal folds. A 4-year-old boy with an inferotemporal retinal
in his right eye.
© 2019 Asia-Pacific Academy of Ophthalmology
Asia-Pacific Journal of Ophthalmology • Volume 8, Number 1, January/February 2019 Persistent Fetal Vasculature

traction and thus leading to tent-shaped retinal detachment (Fig.
6). Macular abnormalities are secondary to tent-shaped or other
tractional retinal detachments. Optic nerve abnormalities are
also found in children with combined PFV. In some children
who are diagnosed with morning glory syndrome, the remnants
of PFV can be identified (Fig. 7). However, the falciform retinal
detachment in retinopathy of prematurity (ROP) and FEVR
FIGURE 5. Ultrasonography of the right eye in a 5-month-old girl. The
axial length is 14.6 mm.
should be distinguished from the tent-shaped retinal detachment
more typical of PFV.
Microphthalmia/Phthisis Bulbi and Buphthalmia
Usually, PFV is accompanied by arrested development of the
eyeball. Microphthalmia and buphthalmia secondary to glaucoma
are severe complications of combined PFV.31
Secondary glaucoma is one of the most common causes of
eventual blindness in children with PFV. The pathogenesis of
secondary glaucoma is varied. It may result from lens swelling
and expansion because of the rupture of the posterior capsule.32
With long-term high intraocular pressure, the corneal and scleral
walls expand, ultimately resulting in buphthalmia. Secondary
glaucoma may also result from inflammatory and iridal
depigmentation or from the concentric dragging of the ciliary
process by the retrolental fibrovascular membrane and sequential
zonular laxity.33
Without treatment, combined PFV can cause corneal
opacification, progressive shallowing of the anterior chamber,
spontaneous intraocular bleeding, and secondary glaucoma. These
complications usually occur suddenly within the first 3 years of
life. The prognosis is quite poor, and sometimes, enucleation is
required because of the pain, uncontrolled intraocular pressure
or phthisis bulbi. Thus, early surgical intervention should be
advocated in the hope of preserving the eye globe, even when no
hope for visual rehabilitation exists.

A B C

FIGURE 6. A, A 1-year-old boy with the primary vitreous located between the posterior lens capsule and the optic papilla in his left eye. The primary
vitreous proliferates and causes local retinal detachment, thus leading to tent-shaped detachment. B, A 5-year-old girl with the primary vitreous,
including the hyaloid artery and proliferative tissues, between the posterior lens capsule and the optic papilla in her left eye. The retina was tracted and
reached the posterior lens capsule; a tent-shaped lesion is located inferior to the optic disc. C, Hyperfluorescence can be found in the tracted retina.

A B

FIGURE 7. The right eye of an 18-month-old girl of whom the disc is enlarged and excavated like a morning glory, with a stalk adherent from the optic
disc to the posterior capsule of lens. A, Fundus picture. B, Fundus fluorescein angiography.

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Chen et al Asia-Pacific Journal of Ophthalmology • Volume 8, Number 1, January/February 2019

IMAGING MODALITIES
Ultrasonography and Color Doppler Imaging
Ultrasonography is widely used in the diagnosis of PFV, as it
provides information on axial length, lens status, and the presence
of a vitreous stalk and retinal detachment when there is a limited
or absent view of the fundus. A-mode ultrasonography is helpful
in revealing a shortened axial length. B-mode ultrasonography
demonstrates the typical lesions occupying the Cloquet canal
between the posterior capsule and the optic disc. The vitreous
after-movement is absent on dynamic scanning in these cases. It
was reported that 92% of cases had typical echographic findings
indicative of PFV34 (Fig. 8).
Combined PFV is found to have typical structural and flow
patterns on color Doppler imaging (CDI)35 and is suggested to
be grouped into 4 types (types I, Y, inverted Y, and X) (Table
1), according to structural and Doppler imaging35 (Figs. 9–12).
Children with different types of PFVs demonstrated different
clinical phenotypes. The axial length is normal in eyes with type
I, but it decreases in the other types, especially in eyes with type
X. However, eyes with combined PFV types Y and X often have
a wide base attached to the posterior surface of the lens, usually
accompanied by ciliary traction/detachment or a dense ciliary
membrane. Eyes with types inverted Y and X have preoptic stalks
with a wide base, whereas eyes with type I have a narrow adhesion
to both the lens and the optic nerve. This new classification
strategy is highly practical for the design of the surgical approach.

Computed Tomography/Magnetic Resonance

FIGURE 8. Ultrasonography of the right eye in a 2-year-old boy with
persistent fetal vasculature. A vitreous stalk is noted, and the axial
length is 15.1 mm.
Imaging
Computed tomography can reveal the ocular and orbital
abnormalities in PFV, such as microphthalmia, buphthalmos,
retrolental fibrosis around the Cloquet canal, and retinal

TABLE 1. Classification of Combined Persistent Fetal Vasculature According to the Structural and Doppler Imaging

Clinical Features
Type Adhesion to the Lens Adhesion to the Optic Nerve Surgical Approach
I Narrow Narrow Posterior (pars plana) approach
Y Wide Narrow Anterior (limbal) approach
Inverted Y Narrow Wide Posterior (pars plana) approach
X Wide Wide Poor prognosis even after surgical treatment; usually no surgical
treatment suggested

A B

FIGURE 9. A 5-month-old boy with an “I”-shaped echo in the left eye, as revealed by color Doppler imaging; blood flow can be detected in the
retrolental band (A) and the optic papilla (B).

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Asia-Pacific Journal of Ophthalmology • Volume 8, Number 1, January/February 2019
FIGURE 10. A 4-month-old boy with a “Y”-shaped echo in his right eye,
as revealed by color Doppler imaging; blood flow can be detected in
the retrolental fibrovascular membrane, dragging the lens into the
vitreous.
FIGURE 11. A 2-year-old girl with an inverted “Y”-shaped echo in her
right eye, as revealed by color Doppler imaging, revealing the traction
on the optic disc and the retina.
detachment (Fig. 13). Hyperflective signals can be detected in
the subretinal area. Intraorbital or ocular calcification is absent
in most cases. The absence of intraocular calcification and
microphthalmos on computed tomography is very important to
differentiate this entity from retinoblastoma.
Optical Coherence Tomography
Optical coherence tomography (OCT) findings are helpful
in showing the dysplastic retina, which is difficult to see
macroscopically. The main anomalous features include posterior
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Persistent Fetal Vasculature
hyaloidal organization, vitreoretinal traction, vitreopapillary
traction, diminished foveal contour, foveal displacement, and
disruption of the ellipsoid zone. Notably, posterior hyaloidal
organization, diminished foveal contour, and disruption of the
ellipsoid zone are associated with worse best-corrected visual
acuity. Macular and vitreomacular interface anomalies have been
identified in all pediatric patients with posterior PFVs imaged
with spectral-domain OCT.36
DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS
Before the 1990s, diagnosing PFV was quite challenging
because of its various clinical manifestations, the difficulty of
comprehensive examinations in pediatric patients, and, most
importantly, the poor understanding of the disease. Fortunately,
the past 2 decades saw rapid progress in imaging techniques,
including CDI, RetCam photography, OCT, and pediatric fundus
fluorescein angiography (FFA), among others, which all made the
diagnosis of PFV more efficient than before (Table 2).
Detection of the hallmark persistent vessels from the optic
disc to the posterior surface by using direct/indirect funduscopy
or the RetCam system, with or without FFA, is the gold standard
for diagnosis. The use of FFA reveals the abnormal vessels and
flow in the affected eye and simultaneously confirms the fundus
of the fellow eye is unremarkable.17,37 The retrolental white
fibrovascular membrane and the traction of ciliary processes are
relatively specific in PFV.17,38 Notably, in some children with
mild PFV, the stalk is quite small and not easy to identify. Figure
14 shows a case of peripheral nonperfusion in the companion eye
revealing traction on the retina in PFV.
Although PFV is a major reason for leukocoria, clinicians
should be aware of differential diagnoses that involve a white pupil
(congenital cataract, retinoblastoma, Norrie disease, ROP, retinal
detachment, and Coats disease). Because of similar retinopathies,
such as falciform retinal folds, the differential diagnosis of PFV
also includes ROP, FEVR,39–41 Norrie disease,42,43 and OPPG
syndrome.44,45
Persistent fetal vasculature can be distinguished from
retinoblastoma, as PFV usually includes monocular involvement,
the coexistence of microphthalmos or cataract, and the absence
of calcification.46 To distinguish PFV from ROP, one needs to
consider characteristics, such as a history of prematurity and
oxygen treatment, along with low body weight. Norrie disease
can be differentiated by its X-linked inheritance, associated
systemic manifestations, and bilaterality.14,47,48 Finally, OPPG
can be differentiated from PFV by its positive family history,
positive genetic test with LRP5 mutation and associated systemic
osteoporotic manifestations, and, importantly, bilaterality.44
TREATMENT
Indications in Surgery for PFV: Past and Present
The treatment of PFV eyes was extremely conservative in
early years. Although vitrectomy was used in PFV patients in the
1980s, the initial goals were cosmetic improvement only; visual
function rescuing was very limited. For many years, posterior
PFV has been considered an exclusion criterion for surgical
treatment, especially in children with tractional retinal detachment
or macular dysplasia.49 The visual outcomes in anterior PFV are
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A B C

FIGURE 12. A 1-year-old boy with an “X”-shaped echo in his right eye (A), as revealed by color Doppler imaging. Blood flow can be detected in the band
extending from the optic disc (B) to the posterior surface of the lens and the retrolental fibrovascular membrane (C).

limited by intraoperative or postoperative complications.

FIGURE 13. A computed tomography image of the right eye of a 5-year-
old boy. A dense band-like shadow adherent to the optic papilla is
revealed (arrow).
However, with the advancements in microsurgical
instruments and technology in recent years and, more importantly,
deeper understanding of this disease, the surgical indications for
PFV have changed.23,50 The surgical outcomes of anterior PFV
have improved dramatically.51–53 Anteby et al18 reported that
6/30 (20%) unilateral anterior PFV eyes received intraocular
lens implantation and obtained a final visual acuity of 20/50 or
better, and 10/30 (33.3%) achieved 20/200 or better. Although
the surgical outcomes in posterior PFV were still limited,
recently, more data supported the consideration of early surgical
intervention for patients with involvement of the macula. The
suggestion is that a period of retinal “physical plasticity”49 extends
to 1 year of age. Bosjolie and Ferrone’s study49 suggested that
surgical intervention for PFV children of 13 months or younger
have potential visual improvement. The management of unilateral
PFV is also extremely challenging.51–54 When the contralateral
eye is normal, overcoming amblyopia and achieving satisfactory
visual acuity in the PFV eye without amblyopia treatment are
very difficult. More reports suggested that only with long-term
postoperative amblyopia treatment could visual function in PFV
patients be significantly improved.50
The indications of PFV surgery expanded in the past 2
decades, including purely anterior PFV, secondary cataract or
retrolenticular fibrous membranes, and posterior or combined
PFV with macular traction and secondary intraocular hemorrhage.
Early lensectomy with or without vitrectomy in children with

TABLE 2. Differential Diagnosis of Persistent Fetal Vasculature

Disease Laterality Family History Systemic Symptoms Genetics Cataract

PFV Unilateral (95%) − − − +, central or nasal
ROP Bilateral − − − Mainly temporal, only in advanced cases
FEVR Bilateral + − FZD4, LRP5, Mainly temporal, only in advanced cases
TSPAN12, NDP
OPPG Bilateral + Osteoporosis LRP5 Mainly temporal, only in advanced cases
FEVR indicates familial exudative vitreoretinopathy; OPPG, osteoporosis–pseudoglioma; PFV, persistent fetal vasculature; ROP, retinopathy of prematurity,
+, positive; −, negative.

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Asia-Pacific Journal of Ophthalmology • Volume 8, Number 1, January/February 2019
FIGURE 14. Fundus fluorescein angiography image of the left eye of
a 1-year-old boy with combined persistent fetal vasculature. Nasal
peripheral nonperfusion is revealed (arrows).
cataract is significantly beneficial. Traction between the lens and
the optic disc, especially macular traction, should be removed,
thus eliminating the risk factors of severe complications, such as
secondary glaucoma, even when a postoperative improvement in
visual function is not expected.55–57 It should be noted that the
prognosis depends on the type and extent of PFV. In isolated
anterior PHPV, a good visual outcome may be achieved when
aphakic correction and amblyopic therapy are successful.
However, the prognosis of purely posterior and combined PFV
is highly limited because of abnormalities in the posterior pole.
Preoperative Evaluation
The extent, timing, and expediency of the surgery must
be tailored to each individual patient before the surgery. A
thorough preoperative evaluation should be performed. The
corneal diameter, anterior chamber depth, and pupil appearance
should be noted. The density of the cataract, integrity of the lens
capsule, presence of lens absorption, liquidation and calcification,
presence of iridal abnormalities and anterior/posterior synechia,
axial length, presence of vitreous lesion, and retinal detachment
should also be noted. Visual axis obscuration, fixation preference,
and pupillary reactions should likewise be evaluated.
Before surgery, a detailed ocular history should be
taken from the parents or guardians. Complete and thorough
communication with the children’s parents is definitely necessary,
as the rehabilitation of the involved eye is dependent largely on
aggressive amblyopia therapy. In 2012, Kozeis et al58 reported a
4-year-old girl with PFV. After a combined lensectomy, posterior
capsulorhexis intraocular lens implantation, and posterior
vitrectomy, with meticulous monitoring of refraction, the final
visual acuity improved to 20/25. Yusuf et al59 reported a boy with
unilateral PFV who underwent cataract extraction, with primary
posterior capsulotomy, anterior vitrectomy, and intraocular
lens implantation, followed by combined trabeculectomy/
trabeculotomy within the first 8 weeks of life. After intensive
optometric treatment, the boy achieved a final visual acuity
of 20/40 (unaided) with no evidence of glaucomatous optic
neuropathy. These 2 cases illustrate that an excellent visual
outcome is possible in unilateral complicated PFV by using an
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Persistent Fetal Vasculature
intensive management approach consisting of early surgical
removal of the cataract and meticulous monitoring of refraction,
visual acuity, and intraocular pressure. Most importantly, the
parents are vitally required to engage in long-term management.
As an intensive optometric and orthoptic input is required for
many years to fight the worsening anisometropic amblyopia,
parents’ engagement may help the children achieve an excellent
final acuity beyond visual maturation.56
SURGICAL PROCEDURES
Surgical Approaches
The anterior (limbal) approach is indicated for anterior
PFV with cataracts and combined PFV type Y or X patients.
The main purpose of the limbal approach is to avoid iatrogenic
breaks of the ora serrata or the peripheral retina. After lens
removal, the opacified posterior lens capsule can be removed with
capsulorhexis; anterior vitrectomy is then performed. Continuous
curvilinear posterior capsulorhexis is completed using an electric
capsulorhexis instrument to remove the posterior capsule of the
lens.
The posterior (pars plana) approach is indicated for
posterior PFV, combined PFV type I, or inverted Y, which are
free from anterior segmental abnormalities. In children, PPV
actually means pars plicata vitrectomy instead of pars plana
vitrectomy. The pars plana in children is very narrow, as it is
not well developed yet. The pars plicata is the preferred site of
vitrectomy. Notably, the location of sclerotomy in cases with
retinal folds should be well designed before the surgery. The
irrigation cannula should be placed away from the meridian
where retinal folds are located. Otherwise, the cannula might
enter the suprachoroidal space, and iatrogenic retina/choroidal
detachment might occur.
Sclerotomy
Sclerotomy is made through the pars plana in elder children
or pars plicata in younger ones, from 1.0 to 3.0 mm posterior to
the limbus, depending on the child’s age at surgery and the extent
of microphthalmia affecting the operative eye.
Residual Stalk Diathermy
In most cases, hemorrhaging from the hyaloid artery in a
fibrovascular stalk may occur and can be resolved by compression.
Stalk amputation can be performed using a vertical pneumatic
scissor without diathermy. The residual stalk regresses with time;
endodiathermy is used if bleeding continues.60
Vitrectomy
Complete vitrectomy is performed around the residual stalk
to remove the sheets of the connected vitreous and residual
hyalocytes around the posterior aspect of the residual stalk. In
some cases, anterior vitrectomy alone is sufficient if no obvious
posterior segment is involved. However, if posterior abnormalities
exist, the epiretinal proliferative membrane around the hyaloid
artery should be removed gently. Iatrogenic retinal breaks should
be avoided.
Posterior Capsulorhexis
Posterior capsule opacification is unavoidable in pediatric
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Chen et al
patients with cataract, so posterior capsulorhexis is necessary
in all PFV-associated cataract eyes. The peripheral anterior
and posterior capsules are reserved for future intraocular lens
implantation.
Postoperative Anti-Inflammation Treatment
As the blood-eye barrier in children has not been fully
developed, incidences of postoperative inflammation are higher.
Thus, anti-inflammation treatment is critical. Usually, the
inflammation is resolved in 1 to 2 weeks. However, in some cases,
a fibrous membrane will lead to pupillary blockage. The pupillary
membrane may remain after the resolution of inflammation and
can be incised with a YAG laser.
Predictors of Surgical Outcomes
Patients with PFV-associated cataract are more likely to
require additional procedures than are patients with congenital
cataract,7 primarily because of lens epithelial cell proliferation
within the visual axis.61 Disease severity, greater posterior
segment involvement, bilaterality, severe microphthalmos, and
posterior disease, which leads to an increased risk of retinal
detachment and glaucoma, adversely affect postoperative
outcomes.20 On the other hand, an unremarkable pupil reflection
and a normal electroretinogram are significant predictors of a
better postoperative visual function. Interestingly, myopia is
associated with favorable visual outcomes in PFV, probably
through the exclusion of microphthalmos and the lesser need for
amblyopia therapy.59
CONCLUSIONS
Persistent fetal vasculature is no longer considered a rare
disease. Thanks to the improvement of new diagnostic strategies,
more and more children with PFV can be identified during their
early life stages. Because of advancements in microinvasive
vitrectomy, the early surgical treatment of PFV has become safer
and more effective, which paves the way for the development of
future new treatment strategies. Moreover, visual rehabilitation
in PFV children requires not only early surgery during the critical
period of visual development but also postoperative management
of amblyopia.
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