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Asia-Pacific Journal of Ophthalmology • Volume 8, Number 1, January/February 2019 Persistent Fetal Vasculature
a number of gene mutations have been shown to cause bilateral and strands, was used in prior literature. These loops and strands
PFV in animal models.9,10 The present literature shows at least are remnants of the anterior TVL, which is the blood supply
15 different genes in knock-out (KO)/transgenic mouse models during lens development of the fetus. Remnants of the capillaries
that exhibit the clinical features of PFV, including CrybetaA3/ may persist as small strands attached to the collarette of the iris,
A1, Lrp5, and Fzd5 KO mouse. However, most of these genes or the membrane-like lesion located in the pupillary area, thus
were notably reported to be associated with familial exudative the term persistent pupillary membrane (Fig. 1). In some cases,
vitreoretinopathy (FEVR). This discrepancy might be explained congenital cataract or retrolental fibrous tissue can be noted in the
by the overlap of clinical phenotypes between FEVR and PFV. 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
CLASSIFICATION compromised as the membrane becomes thick or intact, which
Although the exact prevalence of PFV is still unknown, it causes deprivation amblyopia. In these cases, mydriatic agents,
should not be considered a very rare disease. It accounts for about surgical excision, or laser should be considered to remove the
5% of blindness in childhood in the United States.7 Notably, membrane from the visual axis.
95% of PFV cases are affected unilaterally. Although rare,
bilateral cases have been reported and are usually associated with Mittendorf Dot
congenital syndromes, such as trisomy13,11 trisomy 15,12 Aicardi The Mitterndorf dot is a white dot located on the posterior
syndrome,13 Norrie disease,14 Walker–Warburg syndrome,15 and lens capsule, usually about 0.5 mm nasal to the posterior pole. It
osteoporosis–pseudoglioma (OPPG) syndrome.16 is caused by the incomplete regression of the hyaloid artery. As
Traditionally, PFV could be divided into 3 categories based the well-defined small dot rarely affects vision, no treatment is
on the location of the vascular abnormalities—purely anterior, needed. The Mitterndorf dot can be found in 0.7% to 2.0% of the
purely posterior, and combined PFV.17 Purely anterior PFV is general population.26
relatively common, accounting for approximately 25% of cases
and is characterized by cataracts, retrolental opacity, and, in some Retrolental Membrane
pediatric cases, a shallow anterior chamber and elongation of The retrolental membrane, classically called PHPV, is
ciliary processes. Secondary angle-closure glaucoma, resulting characterized by fibrous membranes located in the retrolental
from a swollen lens, may occur in a few cases. Purely posterior space. It is caused by the failure of regression of the posterior TVL
PFV mainly involves the vitreous and the retina and accounts (Fig. 2). Typically, the retrolental membrane is white or pink,
for 12% of PFV patients. It may manifest as a stalk from the distinguishing it from the yellow exudation found in Coats disease
optic nerve, retinal proliferative membrane, retinal fold, retinal and FEVR, or the snow-white calcification in retinoblastoma. The
detachment, or optic nerve hypoplasia. Combined PFV, involving area of the retrolental membrane varies widely. It may be as small
both the anterior and posterior segments and is the most common as a dot in some cases, or it may occupy the entire posterior surface
type, accounts for about 60% of all cases.6 Of these, 37% to of the lens in others. The lens itself varies from completely clear
46% eventually progress to no-light-perception vision if the to severe opacification. Elongated ciliary processes can be found,
condition is left untreated.18–20 Combined PFV may develop into and these are due to the proliferation and concentric traction of
corneal opacification, secondary angle-closure glaucoma, corneal the remnant posterior TVL (Fig. 3).
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
Persistent Pupillary Membranes FIGURE 1. Persistent pupillary membrane. A 2-year-old boy with a
A more clinically descriptive term, persistent pupillary loops visible pupillary membrane and iris dysplasia in the left eye.
Copyright © 2019 Asia-Pacific Academy of Ophthalmology. Unauthorized reproduction of this article is prohibited.
Chen et al Asia-Pacific Journal of Ophthalmology • Volume 8, Number 1, January/February 2019
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).
Copyright © 2019 Asia-Pacific Academy of Ophthalmology. Unauthorized reproduction of this article is prohibited.
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. should be distinguished from the tent-shaped retinal detachment
6). Macular abnormalities are secondary to tent-shaped or other more typical of PFV.
tractional retinal detachments. Optic nerve abnormalities are
also found in children with combined PFV. In some children Microphthalmia/Phthisis Bulbi and Buphthalmia
who are diagnosed with morning glory syndrome, the remnants Usually, PFV is accompanied by arrested development of the
of PFV can be identified (Fig. 7). However, the falciform retinal eyeball. Microphthalmia and buphthalmia secondary to glaucoma
detachment in retinopathy of prematurity (ROP) and FEVR 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
FIGURE 5. Ultrasonography of the right eye in a 5-month-old girl. The advocated in the hope of preserving the eye globe, even when no
axial length is 14.6 mm. 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.
Copyright © 2019 Asia-Pacific Academy of Ophthalmology. Unauthorized reproduction of this article is prohibited.
Chen et al Asia-Pacific Journal of Ophthalmology • Volume 8, Number 1, January/February 2019
IMAGING MODALITIES of a vitreous stalk and retinal detachment when there is a limited
or absent view of the fundus. A-mode ultrasonography is helpful
Ultrasonography and Color Doppler Imaging in revealing a shortened axial length. B-mode ultrasonography
Ultrasonography is widely used in the diagnosis of PFV, as it demonstrates the typical lesions occupying the Cloquet canal
provides information on axial length, lens status, and the presence 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.
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).
Copyright © 2019 Asia-Pacific Academy of Ophthalmology. Unauthorized reproduction of this article is prohibited.
Asia-Pacific Journal of Ophthalmology • Volume 8, Number 1, January/February 2019 Persistent Fetal Vasculature
Copyright © 2019 Asia-Pacific Academy of Ophthalmology. Unauthorized reproduction of this article is prohibited.
Chen et al Asia-Pacific Journal of Ophthalmology • Volume 8, Number 1, January/February 2019
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).
Copyright © 2019 Asia-Pacific Academy of Ophthalmology. Unauthorized reproduction of this article is prohibited.
Asia-Pacific Journal of Ophthalmology • Volume 8, Number 1, January/February 2019 Persistent Fetal Vasculature
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
FIGURE 14. Fundus fluorescein angiography image of the left eye of breaks of the ora serrata or the peripheral retina. After lens
a 1-year-old boy with combined persistent fetal vasculature. Nasal
peripheral nonperfusion is revealed (arrows).
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
cataract is significantly beneficial. Traction between the lens and lens.
the optic disc, especially macular traction, should be removed, The posterior (pars plana) approach is indicated for
thus eliminating the risk factors of severe complications, such as posterior PFV, combined PFV type I, or inverted Y, which are
secondary glaucoma, even when a postoperative improvement in free from anterior segmental abnormalities. In children, PPV
visual function is not expected.55–57 It should be noted that the actually means pars plicata vitrectomy instead of pars plana
prognosis depends on the type and extent of PFV. In isolated vitrectomy. The pars plana in children is very narrow, as it is
anterior PHPV, a good visual outcome may be achieved when not well developed yet. The pars plicata is the preferred site of
aphakic correction and amblyopic therapy are successful. vitrectomy. Notably, the location of sclerotomy in cases with
However, the prognosis of purely posterior and combined PFV retinal folds should be well designed before the surgery. The
is highly limited because of abnormalities in the posterior pole. irrigation cannula should be placed away from the meridian
where retinal folds are located. Otherwise, the cannula might
Preoperative Evaluation enter the suprachoroidal space, and iatrogenic retina/choroidal
The extent, timing, and expediency of the surgery must detachment might occur.
be tailored to each individual patient before the surgery. A
thorough preoperative evaluation should be performed. The Sclerotomy
corneal diameter, anterior chamber depth, and pupil appearance Sclerotomy is made through the pars plana in elder children
should be noted. The density of the cataract, integrity of the lens or pars plicata in younger ones, from 1.0 to 3.0 mm posterior to
capsule, presence of lens absorption, liquidation and calcification, the limbus, depending on the child’s age at surgery and the extent
presence of iridal abnormalities and anterior/posterior synechia, of microphthalmia affecting the operative eye.
axial length, presence of vitreous lesion, and retinal detachment
should also be noted. Visual axis obscuration, fixation preference, Residual Stalk Diathermy
and pupillary reactions should likewise be evaluated. In most cases, hemorrhaging from the hyaloid artery in a
Before surgery, a detailed ocular history should be fibrovascular stalk may occur and can be resolved by compression.
taken from the parents or guardians. Complete and thorough Stalk amputation can be performed using a vertical pneumatic
communication with the children’s parents is definitely necessary, scissor without diathermy. The residual stalk regresses with time;
as the rehabilitation of the involved eye is dependent largely on endodiathermy is used if bleeding continues.60
aggressive amblyopia therapy. In 2012, Kozeis et al58 reported a
4-year-old girl with PFV. After a combined lensectomy, posterior Vitrectomy
capsulorhexis intraocular lens implantation, and posterior Complete vitrectomy is performed around the residual stalk
vitrectomy, with meticulous monitoring of refraction, the final to remove the sheets of the connected vitreous and residual
visual acuity improved to 20/25. Yusuf et al59 reported a boy with hyalocytes around the posterior aspect of the residual stalk. In
unilateral PFV who underwent cataract extraction, with primary some cases, anterior vitrectomy alone is sufficient if no obvious
posterior capsulotomy, anterior vitrectomy, and intraocular posterior segment is involved. However, if posterior abnormalities
lens implantation, followed by combined trabeculectomy/ exist, the epiretinal proliferative membrane around the hyaloid
trabeculotomy within the first 8 weeks of life. After intensive artery should be removed gently. Iatrogenic retinal breaks should
optometric treatment, the boy achieved a final visual acuity be avoided.
of 20/40 (unaided) with no evidence of glaucomatous optic
neuropathy. These 2 cases illustrate that an excellent visual Posterior Capsulorhexis
outcome is possible in unilateral complicated PFV by using an Posterior capsule opacification is unavoidable in pediatric
Copyright © 2019 Asia-Pacific Academy of Ophthalmology. Unauthorized reproduction of this article is prohibited.
Chen et al Asia-Pacific Journal of Ophthalmology • Volume 8, Number 1, January/February 2019
patients with cataract, so posterior capsulorhexis is necessary cataract surgery in anterior persistent fetal vasculature. J Cataract Refract
in all PFV-associated cataract eyes. The peripheral anterior Surg. 2012;38:849–857.
and posterior capsules are reserved for future intraocular lens 7. Khaliq S, Hameed A, Ismail M, et al. Locus for autosomal recessive
implantation. nonsyndromic persistent hyperplastic primary vitreous. Invest Ophthalmol
Vis Sci. 2001;42:2225–2228.
Postoperative Anti-Inflammation Treatment 8. Morrison DG, Wilson ME, Trivedi RH, et al. Infant Aphakia Treatment
As the blood-eye barrier in children has not been fully Study: effects of persistent fetal vasculature on outcome at 1 year of age. J
developed, incidences of postoperative inflammation are higher. AAPOS. 2011;15:427–431.
Thus, anti-inflammation treatment is critical. Usually, the 9. Grahn BH, Storey ES, McMillan C. Inherited retinal dysplasia and persistent
inflammation is resolved in 1 to 2 weeks. However, in some cases, hyperplastic primary vitreous in miniature Schnauzer dogs. Vet Ophthalmol.
a fibrous membrane will lead to pupillary blockage. The pupillary 2004;7:151–158.
membrane may remain after the resolution of inflammation and 10. Allgoewer I, Pfefferkorn B. Persistent hyperplastic tunica vasculosa lentis
can be incised with a YAG laser. and persistent hyperplastic primary vitreous (PHTVL/PHPV) in two cats.
Vet Ophthalmol. 2001;4:161–164.
Predictors of Surgical Outcomes 11. Allen JC, Venecia G, Opitz JM. Eye findings in the 13 trisomy syndrome.
Patients with PFV-associated cataract are more likely to Eur J Pediatr. 1977;124:179–183.
require additional procedures than are patients with congenital 12. Roch LM 2nd, Petrucci JV, Barber AN. Studies on the development of the
cataract,7 primarily because of lens epithelial cell proliferation eye in the 13-15 trisomy syndrome. Am J Ophthalmol. 1965;60:1067–1074.
within the visual axis.61 Disease severity, greater posterior 13. Carney SH, Brodsky MC, Good WV, et al. Aicardi syndrome: more than
segment involvement, bilaterality, severe microphthalmos, and meets the eye. Surv Ophthalmol. 1993;37:419–424.
posterior disease, which leads to an increased risk of retinal 14. Pendergast SD, Trese MT, Liu X, et al. Study of the Norrie disease gene
detachment and glaucoma, adversely affect postoperative in 2 patients with bilateral persistent hyperplastic primary vitreous. Arch
outcomes.20 On the other hand, an unremarkable pupil reflection Ophthalmol. 1998;116:381–382.
and a normal electroretinogram are significant predictors of a 15. Dobyns WB, Pagon RA, Armstrong D, et al. Diagnostic criteria for Walker-
better postoperative visual function. Interestingly, myopia is Warburg syndrome. Am J Med Genet. 1989;32:195–210.
associated with favorable visual outcomes in PFV, probably 16. Steichen-Gersdorf E, Gassner I, Unsinn K, et al. Persistent hyperplastic
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17. Pollard ZF. Persistent hyperplastic primary vitreous: diagnosis, treatment
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CONCLUSIONS 18. Anteby I, Cohen E, Karshai I, et al. Unilateral persistent hyperplastic
Persistent fetal vasculature is no longer considered a rare primary vitreous: course and outcome. J AAPOS. 2002;6:92–99.
disease. Thanks to the improvement of new diagnostic strategies, 19. Hunt A, Rowe N, Lam A, et al. Outcomes in persistent hyperplastic primary
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vitrectomy, the early surgical treatment of PFV has become safer with or without surgery in persistent fetal vasculature. Ophthalmology.
and more effective, which paves the way for the development of 2010;117:2178–2183.e1–e2.
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in PFV children requires not only early surgery during the critical persistent hyperplastic primary vitreous. Am J Ophthalmol. 1979;88:
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